1LQB

Crystal structure of a hydroxylated HIF-1 alpha peptide bound to the pVHL/elongin-C/elongin-B complex

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations
Protein Modification Annotation

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
C	d1lqbc_	All beta proteins	Prealbumin-like	VHL	VHL	VHL	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d1lqba_	Alpha and beta proteins (a+b)	beta-Grasp (ubiquitin-like)	Ubiquitin-like	Ubiquitin-related	Elongin B	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1lqbb_	Alpha and beta proteins (a+b)	POZ domain	POZ domain	BTB/POZ domain	Elongin C	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
C	SCOP2B Superfamily	VHL	8041313	3000841	SCOP2B (2022-06-29)
A	SCOP2B Superfamily	Ubiquitin-like	8038575	3000157	SCOP2B (2022-06-29)
D	SCOP2B Superfamily	HIF-1alpha pVHL binding region-like	8095959	3002714	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
C	VHL	e1lqbC1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Prealbumin-like	F: VHL	ECOD (1.6)
C	VHL_C	e1lqbC2	A: alpha arrays	X: SOCS box-like (From Topology)	H: SOCS box-like (From Topology)	T: SOCS box-like	F: VHL_C	ECOD (1.6)
A	ubiquitin_2	e1lqbA1	A: a+b two layers	X: beta-Grasp	H: Ubiquitin-related	T: Ubiquitin-like	F: ubiquitin_2	ECOD (1.6)
B	Skp1_POZ_1	e1lqbB1	A: a+b two layers	X: POZ domain (From Topology)	H: POZ domain (From Topology)	T: POZ domain	F: Skp1_POZ_1	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
C	1.10.750.10	Mainly Alpha	Orthogonal Bundle	Elongin C	Chain C, domain 1	CATH (4.3.0)
C	2.60.40.780	Mainly Beta	Sandwich	Immunoglobulin-like	von Hippel-Lindau disease tumour suppressor, beta domain	CATH (4.3.0)
A	3.10.20.90	Alpha Beta	Roll	Ubiquitin-like (UB roll)	Phosphatidylinositol 3-kinase Catalytic Subunit	CATH (4.3.0)
B	3.30.710.10	Alpha Beta	2-Layer Sandwich	Potassium Channel Kv1.1	Chain A	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
C	PF17211	VHL box domain (VHL_C)	VHL box domain	This domain represents the short C-terminal alpha helical domain from the VHL protein [1].	Domain
C	PF01847	VHL beta domain (VHL)	VHL beta domain	VHL forms a ternary complex with the elonginB Swiss:O44226 and elonginC Swiss:O13292 proteins. This complex binds Cul2, which then is involved in regulation of vascular endothelial growth factor Swiss:P15692 mRNA.	Domain
A	PF00240	Ubiquitin family (ubiquitin)	Ubiquitin family	This family contains a number of ubiquitin-like proteins: SUMO (smt3 homologue) (see Swiss:Q02724), Nedd8 (see Swiss:P29595), Elongin B (see Swiss:Q15370), Rub1 (see Swiss:Q9SHE7), and Parkin (see Swiss:O60260). A number of them are thought to carry ...	This family contains a number of ubiquitin-like proteins: SUMO (smt3 homologue) (see Swiss:Q02724), Nedd8 (see Swiss:P29595), Elongin B (see Swiss:Q15370), Rub1 (see Swiss:Q9SHE7), and Parkin (see Swiss:O60260). A number of them are thought to carry a distinctive five-residue motif termed the proteasome-interacting motif (PIM), which may have a biologically significant role in protein delivery to proteasomes and recruitment of proteasomes to transcription sites [5].	Domain
B	PF03931	Skp1 family, tetramerisation domain (Skp1_POZ)	Skp1 family, tetramerisation domain	-	Domain
D	PF11413	Hypoxia-inducible factor-1 (HIF-1)	Hypoxia-inducible factor-1	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
C	von hippel-lindau disease tumor supressor	ubiquitin-like ligase-substrate adaptor activity enzyme-substrate adaptor activity protein-macromolecule adaptor activity molecular adaptor activity transcription corepressor activity transcription regulator activity transcription coregulator activity transcription factor binding protein binding binding DNA-binding transcription factor binding transcription elongation factor activity aminoacyltransferase activity ubiquitin-like protein transferase activity ubiquitin-like ligase-substrate adaptor activity enzyme-substrate adaptor activity protein-macromolecule adaptor activity molecular adaptor activity transcription corepressor activity transcription regulator activity transcription coregulator activity transcription factor binding protein binding binding DNA-binding transcription factor binding transcription elongation factor activity aminoacyltransferase activity ubiquitin-like protein transferase activity catalytic activity, acting on a protein acyltransferase activity transferase activity catalytic activity ubiquitin-protein transferase activity enzyme binding	cellular response to stimulus regulation of signaling regulation of biological process regulation of cellular process response to stimulus negative regulation of signaling signaling negative regulation of response to stimulus biological regulation negative regulation of biological process regulation of signal transduction negative regulation of signal transduction negative regulation of cell communication cell communication regulation of response to stimulus cellular response to stimulus regulation of signaling regulation of biological process regulation of cellular process response to stimulus negative regulation of signaling signaling negative regulation of response to stimulus biological regulation negative regulation of biological process regulation of signal transduction negative regulation of signal transduction negative regulation of cell communication cell communication regulation of response to stimulus negative regulation of cellular process regulation of cell communication cellular process signal transduction regulation of protein stability regulation of biological quality protein stabilization negative regulation of programmed cell death regulation of apoptotic process regulation of programmed cell death cell death negative regulation of apoptotic process apoptotic process programmed cell death response to decreased oxygen levels cellular response to oxygen levels cellular response to hypoxia cellular response to decreased oxygen levels cellular response to chemical stimulus cellular response to stress response to hypoxia response to stress response to chemical response to oxygen levels response to abiotic stimulus organonitrogen compound metabolic process primary metabolic process amyloid fibril formation cellular component organization organic substance metabolic process metabolic process protein metabolic process macromolecule metabolic process cellular component organization or biogenesis supramolecular fiber organization regulation of biosynthetic process cellular biosynthetic process cellular metabolic process regulation of cellular metabolic process regulation of macromolecule metabolic process gene expression macromolecule biosynthetic process regulation of cellular biosynthetic process regulation of gene expression biosynthetic process organic substance biosynthetic process regulation of macromolecule biosynthetic process regulation of metabolic process negative regulation of transcription elongation by RNA polymerase II negative regulation of cellular metabolic process regulation of primary metabolic process negative regulation of RNA biosynthetic process nucleobase-containing compound metabolic process DNA-templated transcription regulation of RNA metabolic process transcription by RNA polymerase II negative regulation of nucleobase-containing compound metabolic process regulation of nucleobase-containing compound metabolic process regulation of DNA-templated transcription elongation negative regulation of DNA-templated transcription regulation of transcription elongation by RNA polymerase II negative regulation of metabolic process negative regulation of cellular biosynthetic process negative regulation of DNA-templated transcription, elongation organic cyclic compound metabolic process negative regulation of macromolecule biosynthetic process organic cyclic compound biosynthetic process regulation of DNA-templated transcription negative regulation of RNA metabolic process transcription elongation by RNA polymerase II RNA metabolic process RNA biosynthetic process nucleic acid biosynthetic process regulation of RNA biosynthetic process negative regulation of macromolecule metabolic process nucleobase-containing compound biosynthetic process nucleic acid metabolic process DNA-templated transcription elongation negative regulation of biosynthetic process protein ubiquitination macromolecule modification post-translational protein modification protein modification process protein modification by small protein conjugation protein modification by small protein conjugation or removal developmental process regulation of developmental process cellular developmental process positive regulation of biological process regulation of cell differentiation positive regulation of developmental process positive regulation of cell differentiation cell differentiation positive regulation of cellular process organonitrogen compound catabolic process catabolic process proteolysis involved in protein catabolic process proteasomal protein catabolic process protein catabolic process proteasome-mediated ubiquitin-dependent protein catabolic process macromolecule catabolic process modification-dependent macromolecule catabolic process modification-dependent protein catabolic process proteolysis ubiquitin-dependent protein catabolic process organic substance catabolic process cell surface receptor signaling pathway via STAT regulation of receptor signaling pathway via JAK-STAT negative regulation of receptor signaling pathway via JAK-STAT cell surface receptor signaling pathway negative regulation of receptor signaling pathway via STAT regulation of receptor signaling pathway via STAT cell surface receptor signaling pathway via JAK-STAT regulation of cellular response to hypoxia regulation of cellular response to stress regulation of response to stress anatomical structure morphogenesis anatomical structure development cell morphogenesis negative regulation of cell population proliferation cell population proliferation regulation of cell population proliferation negative regulation of cellular catabolic process negative regulation of catabolic process autophagy regulation of catabolic process regulation of autophagy cellular catabolic process negative regulation of autophagy regulation of cellular catabolic process process utilizing autophagic mechanism negative regulation of intracellular signal transduction intracellular signal transduction negative regulation of TORC1 signaling regulation of intracellular signal transduction negative regulation of TOR signaling regulation of TORC1 signaling TOR signaling regulation of TOR signaling TORC1 signaling positive regulation of macromolecule biosynthetic process positive regulation of metabolic process positive regulation of macromolecule metabolic process positive regulation of nucleobase-containing compound metabolic process positive regulation of RNA metabolic process positive regulation of DNA-templated transcription positive regulation of cellular metabolic process positive regulation of biosynthetic process positive regulation of RNA biosynthetic process positive regulation of cellular biosynthetic process negative regulation of gene expression regulation of transcription by RNA polymerase II negative regulation of transcription by RNA polymerase II	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol cell periphery plasma membrane membrane intracellular non-membrane-bounded organelle non-membrane-bounded organelle membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol cell periphery plasma membrane membrane intracellular non-membrane-bounded organelle non-membrane-bounded organelle nucleus membrane-enclosed lumen intracellular organelle lumen nucleoplasm organelle lumen nuclear lumen endoplasmic reticulum endomembrane system
A	Elongin B	transcription factor binding transcription coregulator binding transcription corepressor binding protein binding binding ubiquitin protein ligase binding ubiquitin-like protein ligase binding enzyme binding	cellular component organization cellular component biogenesis cellular component assembly protein-containing complex organization protein-containing complex assembly cellular process cellular component organization or biogenesis regulation of cellular process negative regulation of biological process negative regulation of cellular metabolic process nucleobase-containing compound metabolic process post-transcriptional regulation of gene expression organic substance biosynthetic process miRNA metabolic process cellular component organization cellular component biogenesis cellular component assembly protein-containing complex organization protein-containing complex assembly cellular process cellular component organization or biogenesis regulation of cellular process negative regulation of biological process negative regulation of cellular metabolic process nucleobase-containing compound metabolic process post-transcriptional regulation of gene expression organic substance biosynthetic process miRNA metabolic process RNA catabolic process regulation of biological process cellular metabolic process negative regulation of metabolic process regulation of cellular metabolic process primary metabolic process negative regulation of cellular biosynthetic process nucleobase-containing compound catabolic process regulation of gene expression organic cyclic compound metabolic process negative regulation of gene expression biosynthetic process negative regulation of macromolecule biosynthetic process post-transcriptional gene silencing nucleic acid catabolic process regulation of macromolecule biosynthetic process metabolic process macromolecule metabolic process regulation of metabolic process catabolic process cellular biosynthetic process RNA metabolic process macromolecule catabolic process organic substance metabolic process negative regulation of cellular process negative regulation of macromolecule metabolic process regulation of biosynthetic process biological regulation regulation of macromolecule metabolic process miRNA catabolic process gene expression macromolecule biosynthetic process nucleic acid metabolic process organic cyclic compound catabolic process target-directed miRNA degradation regulatory ncRNA-mediated gene silencing regulation of cellular biosynthetic process negative regulation of biosynthetic process ncRNA metabolic process organic substance catabolic process RNA biosynthetic process DNA-templated transcription nucleic acid biosynthetic process transcription by RNA polymerase II transcription initiation at RNA polymerase II promoter nucleobase-containing compound biosynthetic process DNA-templated transcription initiation organic cyclic compound biosynthetic process transcription elongation by RNA polymerase II DNA-templated transcription elongation positive regulation of proteasomal protein catabolic process positive regulation of protein metabolic process proteolysis involved in protein catabolic process positive regulation of biological process proteasome-mediated ubiquitin-dependent protein catabolic process regulation of ubiquitin-dependent protein catabolic process regulation of primary metabolic process positive regulation of metabolic process positive regulation of ubiquitin-dependent protein catabolic process regulation of proteolysis regulation of proteolysis involved in protein catabolic process modification-dependent macromolecule catabolic process proteolysis positive regulation of macromolecule metabolic process protein metabolic process organonitrogen compound catabolic process organonitrogen compound metabolic process regulation of protein metabolic process proteasomal protein catabolic process protein catabolic process regulation of catabolic process positive regulation of protein catabolic process positive regulation of proteolysis involved in protein catabolic process positive regulation of catabolic process regulation of proteasomal ubiquitin-dependent protein catabolic process modification-dependent protein catabolic process positive regulation of proteolysis regulation of proteasomal protein catabolic process positive regulation of proteasomal ubiquitin-dependent protein catabolic process ubiquitin-dependent protein catabolic process regulation of protein catabolic process protein ubiquitination macromolecule modification post-translational protein modification protein modification process protein modification by small protein conjugation protein modification by small protein conjugation or removal	membrane-bounded organelle protein-containing complex intracellular anatomical structure transcription elongation factor complex nucleus membrane-enclosed lumen nuclear protein-containing complex intracellular membrane-bounded organelle cellular anatomical entity elongin complex intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen membrane-bounded organelle protein-containing complex intracellular anatomical structure transcription elongation factor complex nucleus membrane-enclosed lumen nuclear protein-containing complex intracellular membrane-bounded organelle cellular anatomical entity elongin complex intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen organelle nuclear lumen cytoplasm cytosol cytoplasmic ubiquitin ligase complex ubiquitin ligase complex catalytic complex transferase complex intracellular protein-containing complex VCB complex cullin-RING ubiquitin ligase complex Cul5-RING ubiquitin ligase complex Cul2-RING ubiquitin ligase complex
B	Elongin C	transcription factor binding transcription coregulator binding transcription corepressor binding protein binding binding protein-macromolecule adaptor activity molecular adaptor activity	organonitrogen compound catabolic process organonitrogen compound metabolic process catabolic process proteolysis involved in protein catabolic process primary metabolic process protein catabolic process macromolecule catabolic process organic substance metabolic process modification-dependent macromolecule catabolic process modification-dependent protein catabolic process proteolysis ubiquitin-dependent protein catabolic process metabolic process protein metabolic process organonitrogen compound catabolic process organonitrogen compound metabolic process catabolic process proteolysis involved in protein catabolic process primary metabolic process protein catabolic process macromolecule catabolic process organic substance metabolic process modification-dependent macromolecule catabolic process modification-dependent protein catabolic process proteolysis ubiquitin-dependent protein catabolic process metabolic process protein metabolic process macromolecule metabolic process organic substance catabolic process regulation of cellular process negative regulation of biological process negative regulation of cellular metabolic process nucleobase-containing compound metabolic process post-transcriptional regulation of gene expression organic substance biosynthetic process miRNA metabolic process RNA catabolic process regulation of biological process cellular metabolic process negative regulation of metabolic process regulation of cellular metabolic process negative regulation of cellular biosynthetic process nucleobase-containing compound catabolic process regulation of gene expression organic cyclic compound metabolic process negative regulation of gene expression biosynthetic process negative regulation of macromolecule biosynthetic process post-transcriptional gene silencing nucleic acid catabolic process regulation of macromolecule biosynthetic process regulation of metabolic process cellular biosynthetic process RNA metabolic process negative regulation of cellular process negative regulation of macromolecule metabolic process regulation of biosynthetic process biological regulation regulation of macromolecule metabolic process miRNA catabolic process gene expression macromolecule biosynthetic process nucleic acid metabolic process organic cyclic compound catabolic process target-directed miRNA degradation regulatory ncRNA-mediated gene silencing regulation of cellular biosynthetic process negative regulation of biosynthetic process ncRNA metabolic process cellular process RNA biosynthetic process DNA-templated transcription nucleic acid biosynthetic process transcription by RNA polymerase II transcription initiation at RNA polymerase II promoter nucleobase-containing compound biosynthetic process DNA-templated transcription initiation organic cyclic compound biosynthetic process regulation of primary metabolic process regulation of RNA metabolic process regulation of nucleobase-containing compound metabolic process regulation of RNA biosynthetic process regulation of transcription by RNA polymerase II regulation of DNA-templated transcription protein ubiquitination macromolecule modification post-translational protein modification protein modification process protein modification by small protein conjugation protein modification by small protein conjugation or removal	membrane-bounded organelle protein-containing complex intracellular anatomical structure transcription elongation factor complex nucleus membrane-enclosed lumen nuclear protein-containing complex intracellular membrane-bounded organelle cellular anatomical entity elongin complex intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen membrane-bounded organelle protein-containing complex intracellular anatomical structure transcription elongation factor complex nucleus membrane-enclosed lumen nuclear protein-containing complex intracellular membrane-bounded organelle cellular anatomical entity elongin complex intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen organelle nuclear lumen cytoplasm cytosol cullin-RING ubiquitin ligase complex ubiquitin ligase complex catalytic complex transferase complex intracellular protein-containing complex Cul5-RING ubiquitin ligase complex Cul2-RING ubiquitin ligase complex
D	Hypoxia-inducible factor 1 ALPHA	histone deacetylase binding protein binding binding enzyme binding ubiquitin protein ligase binding ubiquitin-like protein ligase binding DNA-binding transcription factor activity, RNA polymerase II-specific transcription regulator activity DNA-binding transcription factor activity DNA-binding transcription activator activity, RNA polymerase II-specific DNA-binding transcription activator activity protein domain specific binding kinase binding protein kinase binding histone deacetylase binding protein binding binding enzyme binding ubiquitin protein ligase binding ubiquitin-like protein ligase binding DNA-binding transcription factor activity, RNA polymerase II-specific transcription regulator activity DNA-binding transcription factor activity DNA-binding transcription activator activity, RNA polymerase II-specific DNA-binding transcription activator activity protein domain specific binding kinase binding protein kinase binding transcription factor binding transcription coregulator binding transcription coactivator binding E-box binding DNA binding sequence-specific DNA binding double-stranded DNA binding nucleic acid binding RNA polymerase II transcription regulatory region sequence-specific DNA binding cis-regulatory region sequence-specific DNA binding sequence-specific double-stranded DNA binding transcription cis-regulatory region binding organic cyclic compound binding transcription regulatory region nucleic acid binding RNA polymerase II cis-regulatory region sequence-specific DNA binding RNA polymerase II-specific DNA-binding transcription factor binding nuclear receptor binding DNA-binding transcription factor binding DNA-binding transcription repressor activity p53 binding Hsp90 protein binding heat shock protein binding molecular function activator activity molecular function regulator activity transcription regulator activator activity protein heterodimerization activity protein dimerization activity	response to stimulus response to activity response to muscle activity multicellular organismal process embryonic epithelial tube formation chordate embryonic development neural fold formation tube formation embryo development multicellular organism development epithelial tube formation primary neural tube formation tube morphogenesis neural fold elevation formation response to stimulus response to activity response to muscle activity multicellular organismal process embryonic epithelial tube formation chordate embryonic development neural fold formation tube formation embryo development multicellular organism development epithelial tube formation primary neural tube formation tube morphogenesis neural fold elevation formation embryo development ending in birth or egg hatching anatomical structure development epithelial tube morphogenesis system development developmental process morphogenesis of an epithelial fold morphogenesis of embryonic epithelium epithelium development neural tube development tube development embryonic morphogenesis morphogenesis of an epithelium nervous system development tissue development tissue morphogenesis anatomical structure morphogenesis neural tube formation anatomical structure formation involved in morphogenesis ossification bone mineralization animal organ development biomineral tissue development cellular developmental process cell development epithelial cell differentiation intestinal epithelial cell maturation columnar/cuboidal epithelial cell development cell differentiation cell maturation digestive system development intestinal epithelial cell differentiation intestinal epithelial cell development developmental maturation digestive tract development epithelial cell development anatomical structure maturation cellular process columnar/cuboidal epithelial cell differentiation epithelial cell maturation columnar/cuboidal epithelial cell maturation ribonucleotide catabolic process pyridine nucleotide catabolic process regulation of cellular process oxoacid metabolic process carbohydrate catabolic process organophosphate catabolic process carbohydrate derivative metabolic process regulation of generation of precursor metabolites and energy nucleoside phosphate catabolic process organic acid metabolic process regulation of primary metabolic process regulation of ATP metabolic process nucleobase-containing compound metabolic process regulation of purine nucleotide catabolic process regulation of small molecule metabolic process glycolytic process regulation of nucleotide metabolic process regulation of phosphate metabolic process phosphate-containing compound metabolic process pyridine-containing compound metabolic process small molecule metabolic process regulation of nucleobase-containing compound metabolic process purine ribonucleotide catabolic process phosphorus metabolic process pyridine nucleotide metabolic process nucleoside phosphate metabolic process organonitrogen compound catabolic process nicotinamide nucleotide metabolic process regulation of carbohydrate catabolic process pyruvate metabolic process regulation of biological process cellular metabolic process purine nucleoside diphosphate metabolic process primary metabolic process regulation of cellular metabolic process purine-containing compound catabolic process nucleotide metabolic process regulation of purine nucleotide metabolic process nucleobase-containing compound catabolic process organic cyclic compound metabolic process nucleoside diphosphate metabolic process purine nucleoside diphosphate catabolic process nucleoside diphosphate catabolic process metabolic process regulation of nucleotide catabolic process regulation of phosphorus metabolic process nucleoside triphosphate metabolic process monocarboxylic acid metabolic process purine ribonucleotide metabolic process regulation of metabolic process pyridine-containing compound catabolic process ribose phosphate metabolic process catabolic process ATP metabolic process organic substance metabolic process purine nucleotide metabolic process ribonucleotide metabolic process nucleobase-containing small molecule metabolic process purine ribonucleoside triphosphate metabolic process ribonucleoside diphosphate metabolic process ribonucleoside diphosphate catabolic process regulation of carbohydrate metabolic process nucleotide catabolic process ribonucleoside triphosphate metabolic process purine nucleoside triphosphate metabolic process carbohydrate metabolic process purine nucleotide catabolic process carbohydrate derivative catabolic process organonitrogen compound metabolic process biological regulation ADP catabolic process carboxylic acid metabolic process regulation of catabolic process generation of precursor metabolites and energy regulation of glycolytic process organic cyclic compound catabolic process purine-containing compound metabolic process ADP metabolic process organophosphate metabolic process purine ribonucleoside diphosphate metabolic process organic substance catabolic process purine ribonucleoside diphosphate catabolic process response to metal ion response to iron ion response to chemical response to inorganic substance positive regulation of macromolecule biosynthetic process DNA-templated transcription positive regulation of metabolic process regulation of RNA metabolic process regulation of miRNA metabolic process organic substance biosynthetic process positive regulation of macromolecule metabolic process miRNA metabolic process positive regulation of nucleobase-containing compound metabolic process positive regulation of RNA metabolic process regulation of gene expression biosynthetic process organic cyclic compound biosynthetic process regulation of macromolecule biosynthetic process macromolecule metabolic process regulation of DNA-templated transcription cellular biosynthetic process positive regulation of DNA-templated transcription positive regulation of biological process RNA metabolic process positive regulation of miRNA transcription positive regulation of cellular metabolic process positive regulation of miRNA metabolic process RNA biosynthetic process nucleic acid biosynthetic process miRNA transcription positive regulation of biosynthetic process positive regulation of cellular process regulation of RNA biosynthetic process regulation of biosynthetic process regulation of macromolecule metabolic process nucleobase-containing compound biosynthetic process gene expression macromolecule biosynthetic process nucleic acid metabolic process regulation of cellular biosynthetic process positive regulation of RNA biosynthetic process ncRNA metabolic process positive regulation of cellular biosynthetic process regulation of miRNA transcription cellular response to stimulus response to oxidative stress cellular response to chemical stimulus cellular response to stress response to stress cellular response to oxidative stress cellular response to chemical stress hemopoiesis embryonic hemopoiesis embryonic organ development mammary gland epithelial cell differentiation mammary gland development gland development epithelial cell differentiation involved in mammary gland alveolus development mammary gland lobule development mammary gland epithelium development mammary gland alveolus development regulation of cytokine production cytokine production regulation of multicellular organismal process regulation of chemokine production positive regulation of gene expression chemokine production positive regulation of chemokine production positive regulation of multicellular organismal process positive regulation of cytokine production negative regulation of metabolic process reactive oxygen species metabolic process negative regulation of biological process negative regulation of cellular metabolic process negative regulation of reactive oxygen species metabolic process negative regulation of cellular process regulation of reactive oxygen species metabolic process camera-type eye morphogenesis sensory organ development eye development visual system development eye morphogenesis sensory organ morphogenesis camera-type eye development iris morphogenesis animal organ morphogenesis sensory system development glandular epithelial cell development glandular epithelial cell maturation glandular epithelial cell differentiation digestive tract morphogenesis regulation of body fluid levels secretion transport establishment of localization body fluid secretion localization lactation regulation of biological quality negative regulation of mesenchymal cell apoptotic process mesenchymal cell apoptotic process negative regulation of programmed cell death regulation of apoptotic process regulation of mesenchymal cell apoptotic process regulation of programmed cell death cell death negative regulation of apoptotic process apoptotic process programmed cell death response to decreased oxygen levels signaling cellular response to oxygen levels cellular response to hypoxia cellular response to decreased oxygen levels cell communication response to hypoxia hypoxia-inducible factor-1alpha signaling pathway response to oxygen levels response to abiotic stimulus signal transduction connective tissue development skeletal system development chondrocyte differentiation cartilage development protein metabolic process glycoprotein metabolic process elastin metabolic process response to oxygen-containing compound response to reactive oxygen species response to organic substance response to cytokine cellular response to interleukin-1 cellular response to cytokine stimulus cellular response to organic substance response to interleukin-1 establishment of organelle localization cellular localization organelle transport along microtubule establishment of mitochondrion localization intracellular transport axonal transport mitochondrion localization establishment of mitochondrion localization, microtubule-mediated axo-dendritic transport mitochondrion transport along microtubule organelle localization establishment of localization in cell microtubule-based transport transport along microtubule cytoskeleton-dependent intracellular transport microtubule-based movement microtubule-based process axonal transport of mitochondrion retina vasculature development in camera-type eye retina development in camera-type eye vasculature development circulatory system development cellular catabolic process positive regulation of ATP metabolic process positive regulation of carbohydrate metabolic process positive regulation of purine nucleotide catabolic process positive regulation of glycolytic process positive regulation of catabolic process positive regulation of phosphate metabolic process positive regulation of nucleotide catabolic process positive regulation of phosphorus metabolic process positive regulation of nucleotide metabolic process positive regulation of purine nucleotide metabolic process regulation of cellular catabolic process positive regulation of cellular catabolic process positive regulation of small molecule metabolic process positive regulation of cell communication positive regulation of signal transduction regulation of chemokine-mediated signaling pathway response to chemokine regulation of signal transduction positive regulation of response to cytokine stimulus cytokine-mediated signaling pathway cellular response to chemokine chemokine-mediated signaling pathway positive regulation of response to stimulus positive regulation of chemokine-mediated signaling pathway regulation of G protein-coupled receptor signaling pathway regulation of signaling cell surface receptor signaling pathway regulation of cytokine-mediated signaling pathway positive regulation of G protein-coupled receptor signaling pathway regulation of response to cytokine stimulus regulation of response to stimulus regulation of cell communication G protein-coupled receptor signaling pathway positive regulation of cytokine-mediated signaling pathway positive regulation of signaling placenta development in utero embryonic development embryonic placenta development regulation of protein modification process regulation of post-translational protein modification regulation of protein metabolic process regulation of protein neddylation macromolecule modification regulation of protein modification by small protein conjugation or removal protein modification process post-translational protein modification protein neddylation protein modification by small protein conjugation protein modification by small protein conjugation or removal negative regulation of miRNA transcription negative regulation of RNA biosynthetic process negative regulation of nucleobase-containing compound metabolic process negative regulation of DNA-templated transcription negative regulation of cellular biosynthetic process negative regulation of macromolecule biosynthetic process negative regulation of RNA metabolic process negative regulation of macromolecule metabolic process negative regulation of biosynthetic process negative regulation of miRNA metabolic process positive regulation of locomotion regulation of cell motility regulation of blood vessel endothelial cell migration endothelial cell migration positive regulation of cell migration positive regulation of epithelial cell migration tissue migration ameboidal-type cell migration positive regulation of cell motility regulation of cell migration blood vessel endothelial cell migration regulation of endothelial cell migration epithelial cell migration cell migration cell motility regulation of epithelial cell migration locomotion positive regulation of blood vessel endothelial cell migration epithelium migration regulation of locomotion positive regulation of endothelial cell migration neuron apoptotic process collagen metabolic process regulation of cytokine production involved in inflammatory response production of molecular mediator involved in inflammatory response defense response cytokine production involved in inflammatory response positive regulation of cytokine production involved in inflammatory response inflammatory response negative regulation of gene expression cell population proliferation positive regulation of cell population proliferation endothelial cell proliferation positive regulation of epithelial cell proliferation regulation of endothelial cell proliferation regulation of cell population proliferation positive regulation of endothelial cell proliferation regulation of epithelial cell proliferation epithelial cell proliferation dopaminergic neuron differentiation generation of neurons neurogenesis neuron differentiation heart development heart morphogenesis outflow tract morphogenesis intracellular iron ion homeostasis inorganic ion homeostasis cellular homeostasis chemical homeostasis homeostatic process intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis neuroblast proliferation neural precursor cell proliferation glucose homeostasis carbohydrate homeostasis intracellular glucose homeostasis regulation of catalytic activity positive regulation of catalytic activity regulation of oxidoreductase activity positive regulation of oxidoreductase activity positive regulation of nitric-oxide synthase activity regulation of molecular function positive regulation of molecular function regulation of monooxygenase activity positive regulation of monooxygenase activity regulation of nitric-oxide synthase activity cellular response to growth factor stimulus cell surface receptor protein tyrosine kinase signaling pathway regulation of vascular endothelial growth factor receptor signaling pathway cellular response to endogenous stimulus enzyme-linked receptor protein signaling pathway response to endogenous stimulus regulation of cellular response to growth factor stimulus vascular endothelial growth factor receptor signaling pathway positive regulation of vascular endothelial growth factor receptor signaling pathway response to growth factor cardiac chamber morphogenesis cardiac ventricle morphogenesis cardiac chamber development cardiac ventricle development regulation of hormone biosynthetic process regulation of hormone metabolic process hormone biosynthetic process positive regulation of hormone metabolic process regulation of hormone levels hormone metabolic process positive regulation of hormone biosynthetic process intracellular oxygen homeostasis positive regulation of signaling receptor activity regulation of signaling receptor activity head development forebrain development brain development pallium development cerebral cortex development central nervous system development telencephalon development negative regulation of signaling negative regulation of intracellular signal transduction intracellular signal transduction negative regulation of cell communication negative regulation of TOR signaling regulation of intracellular signal transduction TOR signaling regulation of TOR signaling negative regulation of response to stimulus negative regulation of signal transduction mesenchyme development mesenchymal cell differentiation epithelial to mesenchymal transition regulation of developmental process regulation of cell differentiation regulation of cell development positive regulation of developmental process positive regulation of cell differentiation regulation of immune system process myeloid cell homeostasis erythrocyte homeostasis regulation of multicellular organismal development regulation of myeloid cell differentiation erythrocyte differentiation regulation of erythrocyte differentiation positive regulation of erythrocyte differentiation multicellular organismal-level homeostasis myeloid cell differentiation immune system process positive regulation of myeloid cell differentiation regulation of hemopoiesis homeostasis of number of cells regulation of transforming growth factor beta production regulation of transforming growth factor beta2 production transforming growth factor beta production transforming growth factor beta2 production intrinsic apoptotic signaling pathway in response to oxidative stress regulation of oxidative stress-induced intrinsic apoptotic signaling pathway negative regulation of neuron apoptotic process regulation of intrinsic apoptotic signaling pathway negative regulation of apoptotic signaling pathway intrinsic apoptotic signaling pathway regulation of oxidative stress-induced neuron intrinsic apoptotic signaling pathway negative regulation of oxidative stress-induced intrinsic apoptotic signaling pathway neuron intrinsic apoptotic signaling pathway in response to oxidative stress apoptotic signaling pathway negative regulation of intrinsic apoptotic signaling pathway negative regulation of oxidative stress-induced neuron intrinsic apoptotic signaling pathway regulation of neuron apoptotic process regulation of apoptotic signaling pathway regulation of growth negative regulation of growth growth aerobic respiration cellular respiration energy derivation by oxidation of organic compounds regulation of cellular respiration regulation of aerobic respiration transcription by RNA polymerase II regulation of transcription by RNA polymerase II positive regulation of transcription by RNA polymerase II pattern specification process embryonic heart tube development embryonic heart tube morphogenesis heart looping determination of bilateral symmetry determination of left/right symmetry specification of symmetry embryonic organ morphogenesis determination of heart left/right asymmetry regionalization left/right pattern formation muscle cell cellular homeostasis regulation of vascular endothelial growth factor production vascular endothelial growth factor production positive regulation of vascular endothelial growth factor production blood vessel morphogenesis angiogenesis blood vessel development wound healing connective tissue replacement involved in inflammatory response wound healing wound healing involved in inflammatory response inflammatory response to wounding tissue remodeling connective tissue replacement response to wounding organic hydroxy compound metabolic process lactate metabolic process stem cell development neural crest cell differentiation neural crest cell development mesenchymal cell migration stem cell differentiation neural crest cell migration response to light stimulus system process learning learning or memory nervous system process visual learning visual behavior cognition associative learning behavior response to radiation protein localization establishment of protein localization nitrogen compound transport cellular macromolecule localization organic substance transport establishment of protein localization to extracellular region protein secretion cellular response to oxygen-containing compound cellular response to carbohydrate stimulus response to glucose insulin secretion involved in cellular response to glucose stimulus hormone secretion protein localization to extracellular region peptide secretion secretion by cell response to monosaccharide response to carbohydrate amide transport signal release cellular response to hexose stimulus cellular response to monosaccharide stimulus cell-cell signaling export from cell cellular response to glucose stimulus peptide transport response to hexose protein transport peptide hormone secretion macromolecule localization hormone transport insulin secretion regulation of localization positive regulation of establishment of protein localization positive regulation of insulin secretion involved in cellular response to glucose stimulus positive regulation of insulin secretion regulation of peptide hormone secretion positive regulation of peptide hormone secretion regulation of protein secretion regulation of secretion positive regulation of protein localization regulation of hormone secretion regulation of protein localization regulation of peptide secretion positive regulation of secretion by cell positive regulation of protein transport regulation of secretion by cell positive regulation of hormone secretion positive regulation of secretion positive regulation of peptide secretion regulation of transport regulation of peptide transport regulation of establishment of protein localization regulation of protein transport positive regulation of protein secretion positive regulation of transport regulation of cellular localization regulation of insulin secretion regulation of insulin secretion involved in cellular response to glucose stimulus hemoglobin biosynthetic process organonitrogen compound biosynthetic process hemoglobin metabolic process regulation of vasculature development regulation of anatomical structure morphogenesis positive regulation of angiogenesis positive regulation of vasculature development regulation of angiogenesis B cell homeostasis leukocyte homeostasis B-1 B cell homeostasis lymphocyte homeostasis positive regulation of organelle organization regulation of cellular component organization positive regulation of cellular component organization macroautophagy autophagy regulation of autophagy positive regulation of autophagy of mitochondrion cellular component organization mitophagy organelle disassembly regulation of autophagy of mitochondrion cellular component organization or biogenesis positive regulation of macroautophagy process utilizing autophagic mechanism mitochondrion organization cellular component disassembly positive regulation of autophagy positive regulation of mitophagy regulation of mitophagy regulation of organelle organization organelle organization regulation of macroautophagy regulation of mitochondrion organization autophagy of mitochondrion negative regulation of thymocyte apoptotic process leukocyte apoptotic process lymphocyte apoptotic process thymocyte apoptotic process negative regulation of leukocyte apoptotic process regulation of T cell apoptotic process regulation of leukocyte apoptotic process regulation of lymphocyte apoptotic process negative regulation of T cell apoptotic process regulation of thymocyte apoptotic process T cell apoptotic process negative regulation of lymphocyte apoptotic process positive regulation of nervous system development regulation of neuroblast proliferation regulation of neurogenesis regulation of nervous system development positive regulation of neurogenesis regulation of neural precursor cell proliferation positive regulation of cell development positive regulation of neural precursor cell proliferation positive regulation of neuroblast proliferation cellular response to virus biological process involved in interspecies interaction between organisms response to other organism response to external stimulus response to external biotic stimulus response to virus response to biotic stimulus negative regulation of biomineral tissue development regulation of biomineral tissue development regulation of bone mineralization negative regulation of bone mineralization negative regulation of developmental process negative regulation of ossification negative regulation of multicellular organismal process regulation of ossification	membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen cellular anatomical entity intracellular membrane-bounded organelle intracellular organelle lumen nuclear speck nuclear body intracellular organelle nucleoplasm organelle lumen organelle nuclear lumen membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen cellular anatomical entity intracellular membrane-bounded organelle intracellular organelle lumen nuclear speck nuclear body intracellular organelle nucleoplasm organelle lumen organelle nuclear lumen cytoplasm cytosol neuron projection cytoplasm axon axon cytoplasm neuron projection cytoplasmic region plasma membrane bounded cell projection cytoplasm plasma membrane bounded cell projection cell projection cilium motile cilium protein-containing complex protein-DNA complex euchromatin chromatin chromosome intracellular non-membrane-bounded organelle non-membrane-bounded organelle RNA polymerase II transcription regulator complex nuclear protein-containing complex transcription regulator complex

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
C	IPR037139	von Hippel-Lindau disease tumour suppressor, alpha domain superfamily	Homologous Superfamily
C	IPR022772	von Hippel-Lindau disease tumour suppressor, beta/alpha domain	Domain
C	IPR024053	von Hippel-Lindau disease tumour suppressor, beta domain	Domain
C	IPR024048	von Hippel-Lindau disease tumour suppressor, alpha domain	Domain
C	IPR036208	VHL superfamily	Homologous Superfamily
C	IPR037140	von Hippel-Lindau disease tumour suppressor, beta domain superfamily	Homologous Superfamily
A	IPR000626	Ubiquitin-like domain	Domain
A	IPR039049	Elongin B	Family
A	IPR029071	Ubiquitin-like domain superfamily	Homologous Superfamily
B	IPR016073	SKP1 component, POZ domain	Domain
B	IPR001232	S-phase kinase-associated protein 1-like	Family
B	IPR039948	Elongin-C	Family
B	IPR011333	SKP1/BTB/POZ domain superfamily	Homologous Superfamily
D	IPR013655	PAS fold-3	Domain
D	IPR001321	Hypoxia-inducible factor-1 alpha	Family
D	IPR035965	PAS domain superfamily	Homologous Superfamily
D	IPR014887	HIF-1 alpha, C-terminal transactivation domain	Domain
D	IPR000014	PAS domain	Domain
D	IPR011598	Myc-type, basic helix-loop-helix (bHLH) domain	Domain
D	IPR001610	PAC motif	Repeat
D	IPR036638	Helix-loop-helix DNA-binding domain superfamily	Homologous Superfamily
D	IPR021537	Hypoxia-inducible factor, alpha subunit-like	Domain
D	IPR013767	PAS fold	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
C	Pharos: P40337	von Hippel-Lindau disease renal cell carcinoma kidney Wilms tumor Chuvash polycythemia familial polycythemia phaeochromocytoma Pheochromocytoma renal carcinoma sarcomatoid renal cell carcinoma kidney cancer microcystic adenoma nonpapillary renal cell carcinoma papillary adenoma clear cell renal carcinoma adenoma von Hippel-Lindau disease renal cell carcinoma kidney Wilms tumor Chuvash polycythemia familial polycythemia phaeochromocytoma Pheochromocytoma renal carcinoma sarcomatoid renal cell carcinoma kidney cancer microcystic adenoma nonpapillary renal cell carcinoma papillary adenoma clear cell renal carcinoma adenoma cancer papillary renal cell carcinoma chromophobe renal cell carcinoma collecting duct carcinoma sarcomatoid carcinoma follicular thyroid adenoma carcinoma paraganglioma erythropoietin polycythemia hemangioblastoma acquired polycythemia vera lung benign neoplasm adrenal medulla cancer benign neoplasm Perlman syndrome extra-adrenal sympathetic paraganglioma undifferentiated carcinoma hemangioma kidney neoplasm neuroendocrine carcinoma benign neoplasm of adrenal gland oligodendroglioma polycythemia diabetes mellitus hereditary Wilms tumor malignant ependymoma adrenal gland pheochromocytoma astrocytic tumor ovarian cancer
A	Pharos: Q15370	acute myeloid leukemia ovarian cancer diabetes mellitus plasma cell myeloma lymphoplasmacytic lymphoma
B	Pharos: Q15369	papillary renal cell carcinoma sarcomatoid renal cell carcinoma collecting duct carcinoma chromophobe renal cell carcinoma renal cell carcinoma astrocytic tumor clear cell renal carcinoma dermatomyositis breast cancer invasive ductal breast carcinoma plasma cell myeloma Conventional (Clear Cell) Renal Cell Carcinoma ovarian cancer
D	Pharos: Q16665	glioblastoma sclerosing cholangitis breast carcinoma hypertensive disorder astrocytic tumor esophageal squamous cell carcinoma hemangioblastoma primary biliary cholangitis Neoplasm Invasiveness thyroid adenoma hyperplasia childhood cerebral astrocytoma pilocytic astrocytoma primary sclerosing cholangitis lung disease glioblastoma sclerosing cholangitis breast carcinoma hypertensive disorder astrocytic tumor esophageal squamous cell carcinoma hemangioblastoma primary biliary cholangitis Neoplasm Invasiveness thyroid adenoma hyperplasia childhood cerebral astrocytoma pilocytic astrocytoma primary sclerosing cholangitis lung disease pulmonary hypertension ductal breast carcinoma in situ malignant pancreatic neoplasm gemistocytic astrocytoma idiopathic pulmonary fibrosis prostate cancer protoplasmic astrocytoma brain ischemia skin cancer fibrillary astrocytoma carcinoma giant cell glioblastoma subependymal giant cell astrocytoma thyroid gland carcinoma anaplastic astrocytoma juvenile pilocytic astrocytoma congestive heart failure sarcomatoid carcinoma skin neoplasm breast ductal adenocarcinoma gastric carcinoma heart failure oligodendroglioma amyotrophic lateral sclerosis ovarian disease kidney disease dermatomyositis diffuse astrocytoma major depressive disorder brain hypoxia - ischemia erythropoietin polycythemia small cell lung carcinoma familial polycythemia cancer spinal cord injury stomach disease cerebral astrocytoma thyroid cancer osteosarcoma cerebrovascular disorder ulcerative colitis pancreatic ductal adenocarcinoma colonic disease juvenile dermatomyositis congenital heart disease B-cell chronic lymphocytic leukemia bipolar disorder breast disease myopathy ovarian cancer cervical cancer undifferentiated carcinoma pancreatic neoplasm inborn carbohydrate metabolic disorder liver disease astrocytoma (excluding glioblastoma) prostate disease diabetes mellitus lymphatic system disease psoriasis Duchenne muscular dystrophy bone disease Emery-Dreifuss muscular dystrophy breast cancer