4CVO

Crystal structure of the N-terminal colied-coil domain of human DNA excision repair protein ERCC-6

External Resource: Annotation

Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	DNA EXCISION REPAIR PROTEIN ERCC-6	protein tyrosine kinase activator activity enzyme regulator activity kinase activator activity enzyme activator activity molecular function activator activity molecular function regulator activity protein kinase activator activity protein kinase regulator activity kinase regulator activity ATP-dependent activity, acting on DNA catalytic activity, acting on DNA catalytic activity, acting on a nucleic acid catalytic activity ATP-dependent activity protein tyrosine kinase activator activity enzyme regulator activity kinase activator activity enzyme activator activity molecular function activator activity molecular function regulator activity protein kinase activator activity protein kinase regulator activity kinase regulator activity ATP-dependent activity, acting on DNA catalytic activity, acting on DNA catalytic activity, acting on a nucleic acid catalytic activity ATP-dependent activity binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding small molecule binding DNA binding nucleic acid binding helicase activity protein-containing complex binding chromatin binding RNA polymerase binding protein binding enzyme binding ATP-dependent chromatin remodeler activity pyrophosphatase activity ATP hydrolysis activity ribonucleoside triphosphate phosphatase activity hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides hydrolase activity hydrolase activity, acting on acid anhydrides	response to light stimulus response to stimulus response to radiation response to abiotic stimulus response to UV response to UV-B response to X-ray response to ionizing radiation response to chemical response to toxic substance cellular response to stimulus transcription-coupled nucleotide-excision repair primary metabolic process DNA metabolic process response to light stimulus response to stimulus response to radiation response to abiotic stimulus response to UV response to UV-B response to X-ray response to ionizing radiation response to chemical response to toxic substance cellular response to stimulus transcription-coupled nucleotide-excision repair primary metabolic process DNA metabolic process nucleic acid metabolic process nucleobase-containing compound metabolic process organic cyclic compound metabolic process organic substance metabolic process cellular response to stress nucleotide-excision repair DNA damage response response to stress metabolic process cellular process macromolecule metabolic process DNA repair regulation of cellular process positive regulation of biological process regulation of cellular response to stress regulation of primary metabolic process positive regulation of response to stimulus positive regulation of metabolic process positive regulation of DNA repair regulation of response to stress positive regulation of macromolecule metabolic process regulation of nucleobase-containing compound metabolic process positive regulation of cellular process regulation of biological process positive regulation of nucleobase-containing compound metabolic process biological regulation regulation of macromolecule metabolic process regulation of DNA repair positive regulation of DNA metabolic process regulation of response to stimulus regulation of metabolic process regulation of DNA metabolic process pyrimidine dimer repair regulation of DNA recombination regulation of double-strand break repair positive regulation of double-strand break repair via homologous recombination recombinational repair double-strand break repair via homologous recombination positive regulation of DNA recombination double-strand break repair DNA recombination positive regulation of double-strand break repair regulation of double-strand break repair via homologous recombination signaling intracellular signal transduction cell communication JNK cascade intracellular signaling cassette MAPK cascade signal transduction base-excision repair developmental process multicellular organism growth multicellular organismal process developmental growth growth positive regulation of macromolecule biosynthetic process DNA-templated transcription regulation of RNA metabolic process transcription by RNA polymerase II organic substance biosynthetic process regulation of transcription by RNA polymerase II cellular metabolic process regulation of cellular metabolic process positive regulation of transcription by RNA polymerase II positive regulation of RNA metabolic process regulation of gene expression biosynthetic process organic cyclic compound biosynthetic process regulation of macromolecule biosynthetic process regulation of DNA-templated transcription positive regulation of DNA-templated transcription cellular biosynthetic process RNA metabolic process positive regulation of cellular metabolic process RNA biosynthetic process nucleic acid biosynthetic process positive regulation of biosynthetic process regulation of RNA biosynthetic process regulation of biosynthetic process nucleobase-containing compound biosynthetic process gene expression macromolecule biosynthetic process regulation of cellular biosynthetic process positive regulation of RNA biosynthetic process positive regulation of cellular biosynthetic process DNA damage checkpoint signaling regulation of cell cycle phase transition negative regulation of biological process negative regulation of cell cycle cell cycle phase transition negative regulation of cellular process negative regulation of cell cycle phase transition cell cycle regulation of cell cycle cell cycle process regulation of cell cycle process DNA integrity checkpoint signaling negative regulation of cell cycle process cell cycle checkpoint signaling signal transduction in response to DNA damage plasma membrane bounded cell projection organization cellular developmental process cell projection organization cell development neuron projection development nervous system development cellular component organization generation of neurons neurogenesis multicellular organism development neuron differentiation cell differentiation neuron development anatomical structure development cellular component organization or biogenesis system development regulation of DNA-templated transcription elongation positive regulation of DNA-templated transcription, elongation DNA-templated transcription elongation double-strand break repair via classical nonhomologous end joining double-strand break repair via nonhomologous end joining negative regulation of double-strand break repair via nonhomologous end joining negative regulation of DNA repair negative regulation of double-strand break repair negative regulation of nucleobase-containing compound metabolic process regulation of double-strand break repair via nonhomologous end joining negative regulation of macromolecule metabolic process negative regulation of response to stimulus negative regulation of metabolic process negative regulation of DNA metabolic process response to oxidative stress response to oxygen-containing compound response to superoxide response to reactive oxygen species response to oxygen radical response to inorganic substance transcription elongation by RNA polymerase II regulation of transcription elongation by RNA polymerase II regulation of DNA-templated transcription initiation transcription initiation at RNA polymerase II promoter positive regulation of DNA-templated transcription initiation positive regulation of transcription initiation by RNA polymerase II DNA-templated transcription initiation regulation of transcription initiation by RNA polymerase II cell death apoptotic signaling pathway intrinsic apoptotic signaling pathway in response to DNA damage apoptotic process intrinsic apoptotic signaling pathway programmed cell death multicellular organismal-level homeostasis homeostatic process anatomical structure homeostasis tissue homeostasis retina homeostasis photoreceptor cell maintenance single strand break repair transcription by RNA polymerase I regulation of transcription by RNA polymerase I positive regulation of transcription by RNA polymerase I response to gamma radiation regulation of transcription by RNA polymerase III positive regulation of transcription by RNA polymerase III transcription by RNA polymerase III transcription elongation by RNA polymerase I rRNA transcription rRNA metabolic process nucleolar large rRNA transcription by RNA polymerase I ncRNA metabolic process DNA protection protein-DNA complex organization protein-containing complex organization chromatin organization chromatin remodeling	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 intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen organelle 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 intracellular organelle lumen intracellular organelle nucleoplasm organelle lumen organelle nuclear lumen nucleolus intracellular non-membrane-bounded organelle non-membrane-bounded organelle ATPase complex B-WICH complex chromosome SWI/SNF superfamily-type complex catalytic complex protein-DNA complex chromatin site of DNA damage

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR049730	SNF2/RAD5-like, C-terminal helicase domain	Domain
A	IPR027417	P-loop containing nucleoside triphosphate hydrolase	Homologous Superfamily
A	IPR001650	Helicase, C-terminal domain-like	Domain
A	IPR014001	Helicase superfamily 1/2, ATP-binding domain	Domain
A	IPR000330	SNF2, N-terminal	Domain
A	IPR038718	SNF2-like, N-terminal domain superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: Q03468	Cockayne syndrome UV-sensitive syndrome de Sanctis-Cacchione syndrome age-related macular degeneration age related macular degeneration 5 cerebrooculofacioskeletal syndrome 1 Cockayne syndrome type 2 microcephaly cataract hearing loss disorder intellectual disability premature aging syndrome COFS syndrome Psychomotor Disorders UV-sensitive syndrome 1 Cockayne syndrome UV-sensitive syndrome de Sanctis-Cacchione syndrome age-related macular degeneration age related macular degeneration 5 cerebrooculofacioskeletal syndrome 1 Cockayne syndrome type 2 microcephaly cataract hearing loss disorder intellectual disability premature aging syndrome COFS syndrome Psychomotor Disorders UV-sensitive syndrome 1 microphthalmia xeroderma pigmentosum arthrogryposis premature ovarian failure 11 retinal degeneration lung benign neoplasm macular degeneration arthrogryposis syndrome microlissencephaly ovarian carcinoma genetic endocrine growth disease Hodgkins lymphoma lung cancer malignant mesothelioma osteogenesis imperfecta ovarian neoplasm premature menopause colorectal cancer primary ovarian failure tuberculosis hydroa vacciniforme leukodystrophy osteosarcoma Cockayne syndrome type 1 distal arthrogryposis

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.