Annotations: 1NHX

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

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d1nhxa1	Alpha and beta proteins (a/b)	PEP carboxykinase-like	PEP carboxykinase-like	PEP carboxykinase C-terminal domain	Cytosolic phosphoenolpyruvate carboxykinase (GTP-hydrolyzing)	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d1nhxa2	Alpha and beta proteins (a/b)	PEP carboxykinase N-terminal domain	PEP carboxykinase N-terminal domain	PEP carboxykinase N-terminal domain	Cytosolic phosphoenolpyruvate carboxykinase (GTP-hydrolyzing)	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)
A	SCOP2 Family	PEPCK-like	8018040	4000841	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	PEPCK/HprK-like	8018041	3001670	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	PEP carboxykinase N-terminal domain	8055993	3001788	SCOP2 (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	PF00821	e1nhxA1	A: a/b three-layered sandwiches	X: P-loop domains-like	H: P-loop domains-related	T: PEP carboxykinase catalytic C-terminal domain	F: PF00821	ECOD (1.6)
A	PF17297	e1nhxA2	A: a/b three-layered sandwiches	X: P-loop domains-like	H: PEP carboxykinase N-terminal domain (From Topology)	T: PEP carboxykinase N-terminal domain	F: PF17297	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.40.449.10	Alpha Beta	3-Layer(aba) Sandwich	Phosphoenolpyruvate Carboxykinase	domain 1	CATH (4.3.0)
A	2.170.8.10	Mainly Beta	Beta Complex	Phosphoenolpyruvate Carboxykinase	domain 2	CATH (4.3.0)
A	3.90.228.20	Alpha Beta	Alpha-Beta Complex	Phosphoenolpyruvate Carboxykinase	domain 3	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF00821	Phosphoenolpyruvate carboxykinase C-terminal P-loop domain (PEPCK_GTP)	Phosphoenolpyruvate carboxykinase C-terminal P-loop domain	Catalyses the formation of phosphoenolpyruvate by decarboxylation of oxaloacetate.	Domain
A	PF17297	Phosphoenolpyruvate carboxykinase N-terminal domain (PEPCK_N)	Phosphoenolpyruvate carboxykinase N-terminal domain	Catalyses the formation of phosphoenolpyruvate by decarboxylation of oxaloacetate.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	PHOSPHOENOLPYRUVATE CARBOXYKINASE, CYTOSOLIC	cation binding manganese ion binding transition metal ion binding ion binding binding metal ion binding small molecule binding organic acid binding anion binding carboxylic acid binding catalytic activity, acting on a protein protein serine kinase activity (using GTP as donor) kinase activity protein kinase activity cation binding manganese ion binding transition metal ion binding ion binding binding metal ion binding small molecule binding organic acid binding anion binding carboxylic acid binding catalytic activity, acting on a protein protein serine kinase activity (using GTP as donor) kinase activity protein kinase activity transferase activity catalytic activity phosphotransferase activity, alcohol group as acceptor transferase activity, transferring phosphorus-containing groups phosphoenolpyruvate carboxykinase activity phosphoenolpyruvate carboxykinase (GTP) activity carboxy-lyase activity lyase activity carbon-carbon lyase activity guanyl nucleotide binding GTP binding nucleotide binding guanyl ribonucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding organic cyclic compound binding purine nucleotide binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding magnesium ion binding GDP binding	organic substance metabolic process tricarboxylic acid metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process small molecule metabolic process metabolic process cellular process organic acid metabolic process response to decreased oxygen levels cellular response to stimulus response to stimulus cellular response to oxygen levels cellular response to hypoxia organic substance metabolic process tricarboxylic acid metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process small molecule metabolic process metabolic process cellular process organic acid metabolic process response to decreased oxygen levels cellular response to stimulus response to stimulus 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 response to organic substance response to raffinose response to oxygen-containing compound cellular response to oxygen-containing compound cellular response to raffinose cellular response to carbohydrate stimulus response to carbohydrate cellular response to organic substance neutral lipid metabolic process neutral lipid biosynthetic process cellular biosynthetic process glycerol biosynthetic process organic hydroxy compound metabolic process lipid biosynthetic process alcohol metabolic process acylglycerol biosynthetic process triglyceride metabolic process triglyceride biosynthetic process organic substance biosynthetic process polyol biosynthetic process pyruvate metabolic process carbohydrate metabolic process polyol metabolic process small molecule biosynthetic process carbohydrate biosynthetic process alditol biosynthetic process glycerol biosynthetic process from pyruvate primary metabolic process glycerol metabolic process biosynthetic process glycerolipid biosynthetic process alditol metabolic process organic hydroxy compound biosynthetic process cellular lipid metabolic process alcohol biosynthetic process glycerolipid metabolic process monocarboxylic acid metabolic process acylglycerol metabolic process lipid metabolic process response to lipid response to retinoic acid cellular response to retinoic acid cellular response to lipid response to ketone cellular response to endogenous stimulus response to hormone response to steroid hormone cellular response to ketone cellular response to steroid hormone stimulus response to organic cyclic compound cellular response to hormone stimulus response to endogenous stimulus cellular response to dexamethasone stimulus response to glucocorticoid cellular response to organic cyclic compound cellular response to glucocorticoid stimulus cellular response to corticosteroid stimulus response to corticosteroid response to dexamethasone response to osmotic stress cellular hypotonic salinity response cellular response to salt stress cellular hypotonic response cellular response to chemical stress cellular response to osmotic stress response to salt stress cellular response to environmental stimulus hypotonic response hypotonic salinity response cellular response to abiotic stimulus response to alcohol response to phorbol 13-acetate 12-myristate cellular response to alcohol cellular response to phorbol 13-acetate 12-myristate monosaccharide metabolic process hexose metabolic process glucose metabolic process developmental process multicellular organismal process epithelium development cellular developmental process gland development animal organ development tissue development liver development epithelial cell differentiation multicellular organism development cell differentiation hepaticobiliary system development anatomical structure development hepatocyte differentiation system development monosaccharide biosynthetic process gluconeogenesis hexose biosynthetic process organonitrogen compound metabolic process protein phosphorylation phosphorylation peptidyl-serine modification peptidyl-serine phosphorylation macromolecule modification peptidyl-amino acid modification phosphate-containing compound metabolic process protein metabolic process macromolecule metabolic process protein modification process phosphorus metabolic process response to acid chemical response to methionine response to organonitrogen compound response to nitrogen compound response to amino acid cellular hyperosmotic salinity response hyperosmotic salinity response hyperosmotic response cellular hyperosmotic response carbohydrate derivative biosynthetic process carbohydrate derivative metabolic process glyceraldehyde-3-phosphate metabolic process aldehyde biosynthetic process organophosphate metabolic process organophosphate biosynthetic process cellular aldehyde metabolic process glyceraldehyde-3-phosphate biosynthetic process dicarboxylic acid metabolic process oxaloacetate metabolic process regulation of cellular process positive regulation of macromolecule biosynthetic process regulation of primary metabolic process nucleobase-containing compound metabolic process positive regulation of metabolic process DNA-templated transcription regulation of RNA metabolic process transcription by RNA polymerase II positive regulation of macromolecule metabolic process regulation of nucleobase-containing compound metabolic process regulation of transcription by RNA polymerase II regulation of biological process positive regulation of nucleobase-containing compound metabolic process regulation of cellular metabolic process positive regulation of transcription by RNA polymerase II positive regulation of RNA metabolic process organic cyclic compound metabolic process regulation of gene expression organic cyclic compound biosynthetic process regulation of macromolecule biosynthetic process regulation of DNA-templated transcription regulation of metabolic process positive regulation of DNA-templated transcription positive regulation of biological process RNA metabolic process positive regulation of cellular metabolic process RNA biosynthetic process nucleic acid biosynthetic process positive regulation of biosynthetic process positive regulation of cellular process regulation of RNA biosynthetic process regulation of biosynthetic process biological regulation 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 positive regulation of cellular biosynthetic process response to cytokine cellular response to interleukin-1 cellular response to cytokine stimulus response to interleukin-1 chemical homeostasis homeostatic process glucose homeostasis carbohydrate homeostasis response to nutrient levels response to starvation catabolic process short-chain fatty acid metabolic process organic acid catabolic process cellular lipid catabolic process cellular catabolic process propionate catabolic process monocarboxylic acid catabolic process carboxylic acid catabolic process propionate metabolic process fatty acid metabolic process short-chain fatty acid catabolic process lipid catabolic process fatty acid catabolic process small molecule catabolic process organic substance catabolic process response to monosaccharide cellular response to hexose stimulus intracellular chemical homeostasis cellular response to monosaccharide stimulus cellular response to glucose stimulus intracellular glucose homeostasis response to hexose cellular homeostasis response to glucose cellular response to nitrogen compound response to peptide hormone cellular response to peptide hormone stimulus response to glucagon cellular response to glucagon stimulus cellular response to organonitrogen compound response to activity regulation of lipid metabolic process positive regulation of lipid biosynthetic process positive regulation of lipid metabolic process regulation of lipid biosynthetic process response to interleukin-6 response to organophosphorus response to purine-containing compound response to cAMP cellular response to cAMP cellular response to tumor necrosis factor response to tumor necrosis factor response to insulin cellular response to fructose stimulus response to fructose cellular response to insulin stimulus cellular response to potassium ion starvation cellular response to nutrient levels cellular response to starvation immunological memory formation process regulation of immune effector process regulation of leukocyte differentiation regulation of leukocyte activation regulation of cell activation mononuclear cell differentiation regulation of cell differentiation lymphocyte differentiation cell activation involved in immune response positive regulation of T cell differentiation regulation of cell development positive regulation of developmental process positive regulation of cell activation positive regulation of immune response T cell activation positive regulation of leukocyte activation regulation of immune system process T cell differentiation positive regulation of immune system process positive regulation of memory T cell differentiation positive regulation of T cell activation immune response positive regulation of cell development immune effector process regulation of response to stimulus regulation of leukocyte cell-cell adhesion regulation of immune response positive regulation of multicellular organismal process leukocyte differentiation regulation of developmental process cell development positive regulation of leukocyte cell-cell adhesion lymphocyte activation leukocyte cell-cell adhesion leukocyte activation positive regulation of cell-cell adhesion regulation of T cell differentiation positive regulation of response to stimulus memory T cell differentiation positive regulation of lymphocyte activation cell adhesion cell-cell adhesion T cell activation involved in immune response positive regulation of hemopoiesis positive regulation of cell differentiation leukocyte activation involved in immune response positive regulation of immune effector process regulation of multicellular organismal process regulation of cell-cell adhesion lymphocyte activation involved in immune response regulation of lymphocyte differentiation regulation of multicellular organismal development hemopoiesis positive regulation of cell adhesion regulation of cell adhesion cell activation regulation of T cell activation T cell differentiation involved in immune response regulation of memory T cell differentiation immune system process regulation of hemopoiesis immunological memory process positive regulation of leukocyte differentiation positive regulation of lymphocyte differentiation regulation of lymphocyte activation biological process involved in interspecies interaction between organisms response to other organism response to external stimulus response to lipopolysaccharide response to biotic stimulus response to bacterium response to external biotic stimulus response to molecule of bacterial origin	extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space cellular anatomical entity vesicle extracellular membrane-bounded organelle intracellular anatomical structure cytoplasm cytosol intracellular organelle extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space cellular anatomical entity vesicle extracellular membrane-bounded organelle intracellular anatomical structure cytoplasm cytosol intracellular organelle endoplasmic reticulum intracellular membrane-bounded organelle endomembrane system

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR008209	Phosphoenolpyruvate carboxykinase, GTP-utilising	Family
A	IPR008210	Phosphoenolpyruvate carboxykinase, N-terminal	Homologous Superfamily
A	IPR035078	Phosphoenolpyruvate carboxykinase, GTP-utilising, N-terminal	Domain
A	IPR035077	Phosphoenolpyruvate carboxykinase, C-terminal P-loop domain	Domain
A	IPR013035	Phosphoenolpyruvate carboxykinase, C-terminal	Homologous Superfamily
A	IPR018091	Phosphoenolpyruvate carboxykinase, GTP-utilising, conserved site	Conserved Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P35558	phosphoenolpyruvate carboxykinase deficiency phosphoenolpyruvate carboxykinase deficiency, cytosolic congestive heart failure Liver Cirrhosis, Experimental obesity disorder psoriasis ulcerative colitis breast fibroadenoma ductal breast carcinoma in situ heart failure herpes zoster lung carcinoma nephrosclerosis schizophrenia atopic eczema phosphoenolpyruvate carboxykinase deficiency phosphoenolpyruvate carboxykinase deficiency, cytosolic congestive heart failure Liver Cirrhosis, Experimental obesity disorder psoriasis ulcerative colitis breast fibroadenoma ductal breast carcinoma in situ heart failure herpes zoster lung carcinoma nephrosclerosis schizophrenia atopic eczema Emery-Dreifuss muscular dystrophy invasive ductal breast carcinoma malignant colon neoplasm pituitary cancer breast carcinoma hepatocellular carcinoma lung cancer

Structure Motif Annotation: Mechanism and Catalytic Site Atlas M-CSA Database Homepage

Chains	Enzyme Name	Description	Catalytic Residues
A	phosphoenolpyruvate carboxykinase (GTP) M-CSA #863	GTP-utilising phosphoenolpyruvate carboxykinase (PEPCK) catalyses the first committed (rate-limiting) step in hepatic gluconeogenesis, namely the reversible decarboxylation of oxaloacetate to phosphoenolpyruvate (PEP) and carbon dioxide using GTP as a source of phosphate.	Defined by 8 residues: ARG:A-90 [auth A-87]TYR:A-238 [auth A-235]LYS:A-247 [auth A-244]HIS:A-267 [auth A-264]SER:A-289 [auth A-286]CYS:A-291 [auth A-288]ASP:A-314 [auth A-311]ARG:A-408 [auth A-405] \| Explore in 3D: M-CSA Motif Definition Search M-CSA Motif EC: 4.1.1.32 (PDB Primary Data) Search M-CSA Motif + EC 4.1.1.32

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM133198.