8RUT | pdb_00008rut

HIF prolyl-hydroxylase-2 (PHD2) T387V variant bound to Fe(III), 2-oxoglutarate (2OG) and Hypoxia-inducible Factor-2alpha (HIF-2alpha)

External Resource: Annotation

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

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF13640	2OG-Fe(II) oxygenase superfamily (2OG-FeII_Oxy_3)	2OG-Fe(II) oxygenase superfamily	This family contains members of the 2-oxoglutarate (2OG) and Fe(II)-dependent oxygenase superfamily [1].	Domain
B	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
A	Egl nine homolog 1	cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding peptidyl-proline 4-dioxygenase activity oxidoreductase activity catalytic activity, acting on a protein oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen peptidyl-proline dioxygenase activity catalytic activity cation binding transition metal ion binding iron ion binding ion binding binding ferrous iron binding metal ion binding small molecule binding peptidyl-proline 4-dioxygenase activity oxidoreductase activity catalytic activity, acting on a protein oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen peptidyl-proline dioxygenase activity catalytic activity 2-oxoglutarate-dependent dioxygenase activity dioxygenase activity organic acid binding carbohydrate binding vitamin binding anion binding monosaccharide binding carboxylic acid binding heterocyclic compound binding L-ascorbic acid binding hypoxia-inducible factor-proline dioxygenase activity zinc ion binding protein binding enzyme binding	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 developmental process multicellular organismal process regulation of multicellular organismal development regulation of biological process 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 developmental process multicellular organismal process regulation of multicellular organismal development regulation of biological process regulation of developmental process blood vessel morphogenesis tube development biological regulation angiogenesis vasculature development regulation of vasculature development circulatory system development anatomical structure morphogenesis multicellular organism development tube morphogenesis regulation of angiogenesis anatomical structure formation involved in morphogenesis anatomical structure development regulation of multicellular organismal process regulation of anatomical structure morphogenesis blood vessel development system development heart development cardiac chamber morphogenesis animal organ development heart morphogenesis ventricular septum development cardiac septum development cardiac septum morphogenesis ventricular septum morphogenesis cardiac chamber development animal organ morphogenesis cardiac ventricle development regulation of cellular process negative regulation of DNA-binding transcription factor activity RNA metabolic process regulation of primary metabolic process nucleobase-containing compound metabolic process RNA biosynthetic process nucleic acid biosynthetic process DNA-templated transcription regulation of RNA metabolic process regulation of nucleobase-containing compound metabolic process regulation of RNA biosynthetic process regulation of biosynthetic process negative regulation of molecular function primary metabolic process regulation of macromolecule metabolic process nucleobase-containing compound biosynthetic process gene expression macromolecule biosynthetic process nucleic acid metabolic process regulation of molecular function regulation of gene expression biosynthetic process regulation of DNA-binding transcription factor activity cellular process metabolic process regulation of macromolecule biosynthetic process regulation of DNA-templated transcription macromolecule metabolic process regulation of metabolic process cardiac muscle tissue development tissue development muscle tissue morphogenesis striated muscle tissue development muscle structure development tissue morphogenesis cardiac muscle tissue morphogenesis muscle organ development muscle tissue development muscle organ morphogenesis placenta development chordate embryonic development labyrinthine layer development embryo development in utero embryonic development embryonic placenta development embryo development ending in birth or egg hatching embryonic organ development 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 regulation protein catabolic process at synapse catabolic process regulation of catabolic process regulation protein catabolic process at postsynapse neuron apoptotic process regulation of apoptotic process regulation of programmed cell death cell death apoptotic process programmed cell death regulation of neuron apoptotic process negative regulation of hydrolase activity regulation of catalytic activity regulation of hydrolase activity negative regulation of cyclic-nucleotide phosphodiesterase activity negative regulation of catalytic activity heart trabecula formation heart trabecula morphogenesis trabecula formation trabecula morphogenesis intracellular oxygen homeostasis positive regulation of DNA-templated transcription positive regulation of biological process positive regulation of macromolecule biosynthetic process positive regulation of metabolic process transcription by RNA polymerase II positive regulation of biosynthetic process positive regulation of macromolecule metabolic process positive regulation of cellular process regulation of transcription by RNA polymerase II positive regulation of nucleobase-containing compound metabolic process positive regulation of transcription by RNA polymerase II positive regulation of RNA metabolic process positive regulation of RNA biosynthetic process regulation of cellular component organization regulation of modification of synaptic structure cellular component organization regulation of synapse organization cell junction organization regulation of synapse structure or activity regulation of modification of postsynaptic structure synapse organization cellular component organization or biogenesis regulation of biological quality modification of synaptic structure modification of postsynaptic structure response to nitric oxide response to oxygen-containing compound response to nitrogen compound	cellular anatomical structure intracellular anatomical structure cytoplasm cytosol membrane-bounded organelle intracellular organelle organelle intracellular membrane-bounded organelle synapse cell junction glutamatergic synapse postsynaptic density postsynapse neuron to neuron synapse cellular anatomical structure intracellular anatomical structure cytoplasm cytosol membrane-bounded organelle intracellular organelle organelle intracellular membrane-bounded organelle synapse cell junction glutamatergic synapse postsynaptic density postsynapse neuron to neuron synapse postsynaptic specialization asymmetric synapse nucleus
B	Hypoxia-inducible Factor-2alpha	transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding protein binding binding DNA-binding transcription factor 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 transcription regulatory region nucleic acid binding transcription factor binding RNA polymerase II-specific DNA-binding transcription factor binding protein binding binding DNA-binding transcription factor 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 transcription regulatory region nucleic acid binding RNA polymerase II cis-regulatory region sequence-specific DNA binding protein heterodimerization activity protein dimerization activity DNA-binding transcription factor activity, RNA polymerase II-specific transcription regulator activity DNA-binding transcription factor activity transcription coregulator binding transcription coactivator binding DNA-binding transcription activator activity, RNA polymerase II-specific DNA-binding transcription activator activity	heart process multicellular organismal process system process regulation of biological process regulation of heart contraction regulation of system process biological regulation circulatory system process regulation of blood circulation regulation of multicellular organismal process regulation of biological quality heart contraction blood circulation regulation of heart rate heart process multicellular organismal process system process regulation of biological process regulation of heart contraction regulation of system process biological regulation circulatory system process regulation of blood circulation regulation of multicellular organismal process regulation of biological quality heart contraction blood circulation regulation of heart rate multicellular organismal-level homeostasis chemical homeostasis homeostatic process multicellular organismal-level chemical homeostasis surfactant homeostasis developmental process blood vessel morphogenesis tube development angiogenesis vasculature development circulatory system development anatomical structure morphogenesis multicellular organism development tube morphogenesis anatomical structure formation involved in morphogenesis anatomical structure development blood vessel development system development muscle structure development cellular developmental process cell differentiation cellular process myoblast differentiation cell fate commitment myoblast fate commitment 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 animal organ development respiratory tube development lung development respiratory system development multicellular organismal-level iron ion homeostasis inorganic ion homeostasis monoatomic ion homeostasis monoatomic cation homeostasis placenta development chordate embryonic development embryo development in utero embryonic development embryonic placenta development embryo development ending in birth or egg hatching embryonic organ development regulation of cellular process regulation of protein modification process regulation of post-translational protein modification regulation of protein metabolic process primary metabolic process regulation of macromolecule metabolic process regulation of primary metabolic process regulation of protein neddylation macromolecule modification metabolic process protein metabolic process macromolecule metabolic process regulation of protein modification by small protein conjugation or removal protein modification process post-translational protein modification regulation of metabolic process protein neddylation protein modification by small protein conjugation protein modification by small protein conjugation or removal signaling signal transduction cell communication positive regulation of biological process adaptive thermogenesis positive regulation of metabolic process regulation of cold-induced thermogenesis cold-induced thermogenesis temperature homeostasis positive regulation of cold-induced thermogenesis positive regulation of multicellular organismal process positive regulation of cellular process blood vessel remodeling tissue remodeling amine metabolic process catecholamine metabolic process phenol-containing compound metabolic process catechol-containing compound metabolic process norepinephrine metabolic process biogenic amine metabolic process mRNA transcription by RNA polymerase II RNA metabolic process nucleobase-containing compound biosynthetic process gene expression mRNA transcription mRNA metabolic process macromolecule biosynthetic process nucleic acid metabolic process nucleobase-containing compound metabolic process RNA biosynthetic process DNA-templated transcription nucleic acid biosynthetic process transcription by RNA polymerase II biosynthetic process sensory perception of light stimulus sensory perception nervous system process visual perception hemopoiesis cell development erythrocyte differentiation myeloid cell differentiation immune system process homeostasis of number of cells myeloid cell homeostasis erythrocyte homeostasis 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 biosynthetic process regulation of gene expression regulation of macromolecule biosynthetic process regulation of DNA-templated transcription response to oxidative stress cellular component organization organelle organization cellular component organization or biogenesis mitochondrion organization positive regulation of DNA-templated transcription positive regulation of macromolecule biosynthetic process positive regulation of biosynthetic process positive regulation of macromolecule metabolic process positive regulation of nucleobase-containing compound metabolic process positive regulation of transcription by RNA polymerase II positive regulation of RNA metabolic process positive regulation of RNA biosynthetic process epithelium development developmental maturation tissue development epithelial cell differentiation epithelial cell development anatomical structure maturation epithelial cell maturation cell maturation	membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle intracellular organelle lumen nuclear speck nuclear body intracellular organelle cellular anatomical structure nucleoplasm organelle lumen organelle nuclear lumen membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle intracellular organelle lumen nuclear speck nuclear body intracellular organelle cellular anatomical structure nucleoplasm organelle lumen organelle nuclear lumen cytoplasm cytosol protein-containing complex transcription regulator complex membraneless organelle intracellular membraneless organelle chromatin chromosome

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR006620	Prolyl 4-hydroxylase, alpha subunit	Domain
A	IPR002893	Zinc finger, MYND-type	Domain
A	IPR044862	Prolyl 4-hydroxylase alpha subunit, Fe(2+) 2OG dioxygenase domain	Domain
A	IPR051559	Hypoxia-inducible factor prolyl hydroxylases	Family
A	IPR005123	Oxoglutarate/iron-dependent dioxygenase domain	Domain
B	IPR021537	Hypoxia-inducible factor, alpha subunit-like	Domain
B	IPR014887	HIF-1 alpha, C-terminal transactivation domain	Domain
B	IPR013767	PAS fold	Domain
B	IPR001067	Nuclear translocator	Family
B	IPR000014	PAS domain	Domain
B	IPR011598	Myc-type, basic helix-loop-helix (bHLH) domain	Domain
B	IPR035965	PAS domain superfamily	Homologous Superfamily
B	IPR001610	PAC motif	Repeat
B	IPR036638	Helix-loop-helix DNA-binding domain superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: Q9GZT9	erythrocytosis, familial, 3 familial polycythemia polycythemia ovarian cancer Refractory anemia (clinical) toxic encephalopathy acquired polycythemia vera facioscapulohumeral muscular dystrophy glioblastoma malignant glioma non-small cell lung carcinoma pancreatic ductal adenocarcinoma adrenal medulla cancer atypical teratoid rhabdoid tumor large cell medulloblastoma erythrocytosis, familial, 3 familial polycythemia polycythemia ovarian cancer Refractory anemia (clinical) toxic encephalopathy acquired polycythemia vera facioscapulohumeral muscular dystrophy glioblastoma malignant glioma non-small cell lung carcinoma pancreatic ductal adenocarcinoma adrenal medulla cancer atypical teratoid rhabdoid tumor large cell medulloblastoma osteosarcoma secondary polycythemia benign neoplasm of adrenal gland erythropoietin polycythemia lung cancer myopathy
B	Pharos: Q99814	erythrocytosis, familial, 4 renal cell carcinoma familial polycythemia sarcomatoid renal cell carcinoma chromophobe renal cell carcinoma papillary renal cell carcinoma Chromosome Aberrations collecting duct carcinoma invasive ductal breast carcinoma lung cancer nasopharyngeal carcinoma oligodendroglioma acquired polycythemia vera pancreatic intraductal papillary-mucinous neoplasm atypical teratoid rhabdoid tumor erythrocytosis, familial, 4 renal cell carcinoma familial polycythemia sarcomatoid renal cell carcinoma chromophobe renal cell carcinoma papillary renal cell carcinoma Chromosome Aberrations collecting duct carcinoma invasive ductal breast carcinoma lung cancer nasopharyngeal carcinoma oligodendroglioma acquired polycythemia vera pancreatic intraductal papillary-mucinous neoplasm atypical teratoid rhabdoid tumor primitive neuroectodermal tumor von Hippel-Lindau disease clear cell renal carcinoma ductal breast carcinoma in situ large cell medulloblastoma lung carcinoma MALT lymphoma osteosarcoma adrenal cortex carcinoma renal carcinoma breast carcinoma secondary polycythemia lung adenocarcinoma malignant mesothelioma medulloblastoma non-small cell lung carcinoma ovarian cancer adrenal medulla cancer polycythemia benign neoplasm of adrenal gland tuberculosis chronic obstructive pulmonary disease erythropoietin polycythemia

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), 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 R01GM157729.