8RE8

Aspartyl/Asparaginyl beta-hydroxylase (AspH) R688Q variant in complex with Mn, (3R)-methyl-2-oxoglutarate and a Factor X derived peptide fragment

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	Aspartyl/asparaginyl beta-hydroxylase	cation binding ion binding binding calcium ion binding metal ion binding small molecule binding structural molecule activity structural constituent of muscle electron transfer activity protein binding transmembrane transporter binding oxidoreductase activity catalytic activity, acting on a protein oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen cation binding ion binding binding calcium ion binding metal ion binding small molecule binding structural molecule activity structural constituent of muscle electron transfer activity protein binding transmembrane transporter binding oxidoreductase activity catalytic activity, acting on a protein oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen peptidyl-aspartic acid 3-dioxygenase activity catalytic activity 2-oxoglutarate-dependent dioxygenase activity dioxygenase activity	developmental process multicellular organism development multicellular organismal process anatomical structure development pattern specification process regulation of biological process regulation of cellular process biological regulation regulation of cell communication cellular process regulation of cell communication by electrical coupling cell communication by electrical coupling cell communication activation of cysteine-type endopeptidase activity developmental process multicellular organism development multicellular organismal process anatomical structure development pattern specification process regulation of biological process regulation of cellular process biological regulation regulation of cell communication cellular process regulation of cell communication by electrical coupling cell communication by electrical coupling cell communication activation of cysteine-type endopeptidase activity regulation of catalytic activity positive regulation of protein metabolic process positive regulation of peptidase activity positive regulation of biological process protein maturation regulation of primary metabolic process positive regulation of molecular function positive regulation of metabolic process regulation of proteolysis proteolysis regulation of endopeptidase activity positive regulation of macromolecule metabolic process protein metabolic process positive regulation of cellular process zymogen activation regulation of cysteine-type endopeptidase activity regulation of protein metabolic process regulation of peptidase activity positive regulation of catalytic activity primary metabolic process regulation of macromolecule metabolic process gene expression macromolecule biosynthetic process regulation of molecular function positive regulation of hydrolase activity biosynthetic process positive regulation of cysteine-type endopeptidase activity regulation of hydrolase activity positive regulation of endopeptidase activity positive regulation of proteolysis protein processing metabolic process macromolecule metabolic process regulation of metabolic process regulation of calcium ion transmembrane transporter activity regulation of calcium ion transport regulation of localization intracellular calcium ion homeostasis negative regulation of biological process regulation of inositol 1,4,5-trisphosphate-sensitive calcium-release channel activity calcium ion transport localization monoatomic cation homeostasis monoatomic cation transport intracellular monoatomic ion homeostasis regulation of release of sequestered calcium ion into cytosol regulation of monoatomic ion transport inorganic ion transmembrane transport establishment of localization regulation of cation channel activity inorganic cation transmembrane transport maintenance of location transmembrane transport release of sequestered calcium ion into cytosol regulation of transmembrane transport chemical homeostasis cellular localization inorganic ion homeostasis transport homeostatic process monoatomic ion transmembrane transport regulation of transmembrane transporter activity calcium ion homeostasis regulation of monoatomic ion transmembrane transport negative regulation of sequestering of calcium ion metal ion transport maintenance of location in cell regulation of transporter activity regulation of calcium ion transmembrane transport monoatomic cation transmembrane transport intracellular monoatomic cation homeostasis monoatomic ion homeostasis negative regulation of cellular process monoatomic ion transport intracellular chemical homeostasis regulation of metal ion transport regulation of sequestering of calcium ion regulation of monoatomic ion transmembrane transporter activity calcium ion transmembrane import into cytosol regulation of transport sequestering of calcium ion cellular homeostasis regulation of monoatomic cation transmembrane transport calcium ion transmembrane transport anatomical structure morphogenesis head development face morphogenesis head morphogenesis face development body morphogenesis sarcoplasmic reticulum calcium ion transport release of sequestered calcium ion into cytosol by sarcoplasmic reticulum release of sequestered calcium ion into cytosol by endoplasmic reticulum regulation of release of sequestered calcium ion into cytosol by sarcoplasmic reticulum cell population proliferation regulation of ryanodine-sensitive calcium-release channel activity limb development appendage development appendage morphogenesis limb morphogenesis cellular response to stimulus cellular response to metal ion cellular response to chemical stimulus response to stimulus response to metal ion response to chemical cellular response to calcium ion response to calcium ion cellular component disassembly regulation of cellular component organization regulation of protein depolymerization protein-containing complex organization protein-containing complex disassembly regulation of protein-containing complex disassembly cellular component organization cellular component organization or biogenesis protein depolymerization regulation of protein stability regulation of biological quality positive regulation of cation transmembrane transport positive regulation of calcium ion transport positive regulation of monoatomic ion transport positive regulation of monoatomic ion transmembrane transport positive regulation of cation channel activity positive regulation of calcium ion transmembrane transporter activity positive regulation of calcium ion transmembrane transport positive regulation of transmembrane transport positive regulation of release of sequestered calcium ion into cytosol positive regulation of ryanodine-sensitive calcium-release channel activity positive regulation of transport positive regulation of transporter activity positive regulation of ion transmembrane transporter activity negative regulation of cell population proliferation regulation of cell population proliferation response to organophosphorus response to oxygen-containing compound response to nitrogen compound response to ATP response to purine-containing compound response to organic cyclic compound regulation of calcium ion transport into cytosol positive regulation of cytosolic calcium ion concentration positive regulation of calcium ion transport into cytosol regulation of cellular localization calcium ion transport into cytosol system process regulation of heart contraction regulation of system process cardiac muscle contraction intracellular signal transduction circulatory system process intracellular signaling cassette signal transduction regulation of cardiac muscle contraction muscle contraction regulation of blood circulation regulation of striated muscle contraction calcium-mediated signaling regulation of muscle contraction heart process signaling regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ion striated muscle contraction regulation of multicellular organismal process blood circulation regulation of cardiac muscle contraction by calcium ion signaling muscle system process regulation of muscle system process heart contraction positive regulation of DNA-templated transcription RNA metabolic process positive regulation of macromolecule biosynthetic process nucleobase-containing compound metabolic process RNA biosynthetic process nucleic acid biosynthetic process DNA-templated transcription regulation of RNA metabolic process positive regulation of biosynthetic process regulation of nucleobase-containing compound metabolic process regulation of RNA biosynthetic process regulation of biosynthetic process positive regulation of nucleobase-containing compound metabolic process nucleobase-containing compound biosynthetic process nucleic acid metabolic process positive regulation of RNA metabolic process regulation of gene expression positive regulation of RNA biosynthetic process regulation of macromolecule biosynthetic process regulation of DNA-templated transcription macromolecule modification peptidyl-amino acid modification protein hydroxylation peptidyl-aspartic acid modification protein modification process peptidyl-aspartic acid hydroxylation activation of store-operated calcium channel activity regulation of store-operated calcium channel activity positive regulation of store-operated calcium channel activity protein localization establishment of protein localization nitrogen compound transport regulation of protein localization positive regulation of establishment of protein localization positive regulation of protein transport cellular macromolecule localization establishment of localization in cell positive regulation of intracellular transport regulation of intracellular transport regulation of establishment of protein localization intracellular transport regulation of protein transport protein transport macromolecule localization positive regulation of protein localization regulation of intracellular protein transport positive regulation of intracellular protein transport intracellular protein transport roof of mouth development detection of stimulus detection of chemical stimulus detection of calcium ion	membrane-bounded organelle intracellular anatomical structure organelle subcompartment membrane intracellular membrane-bounded organelle endomembrane system sarcoplasm endoplasmic reticulum subcompartment intracellular organelle cellular anatomical structure bounding membrane of organelle organelle nuclear outer membrane-endoplasmic reticulum membrane network endoplasmic reticulum membrane-bounded organelle intracellular anatomical structure organelle subcompartment membrane intracellular membrane-bounded organelle endomembrane system sarcoplasm endoplasmic reticulum subcompartment intracellular organelle cellular anatomical structure bounding membrane of organelle organelle nuclear outer membrane-endoplasmic reticulum membrane network endoplasmic reticulum sarcoplasmic reticulum sarcoplasmic reticulum membrane cytoplasm junctional sarcoplasmic reticulum membrane organelle membrane endoplasmic reticulum membrane cell periphery plasma membrane membrane-enclosed lumen sarcoplasmic reticulum lumen intracellular organelle lumen endoplasmic reticulum lumen organelle lumen protein-containing complex transmembrane transporter complex membrane protein complex cation channel complex transporter complex calcium channel complex monoatomic ion channel complex cell cortex cortical endoplasmic reticulum endoplasmic reticulum tubular network
B	Coagulation factor X	cation binding ion binding binding calcium ion binding metal ion binding small molecule binding peptidase activity catalytic activity, acting on a protein serine hydrolase activity serine-type peptidase activity hydrolase activity serine-type endopeptidase activity catalytic activity endopeptidase activity cation binding ion binding binding calcium ion binding metal ion binding small molecule binding peptidase activity catalytic activity, acting on a protein serine hydrolase activity serine-type peptidase activity hydrolase activity serine-type endopeptidase activity catalytic activity endopeptidase activity phospholipid binding lipid binding	multicellular organismal process regulation of body fluid levels wound healing response to stimulus hemostasis biological regulation blood coagulation response to stress coagulation regulation of biological quality response to wounding cellular response to stimulus positive regulation of cell communication regulation of cellular process multicellular organismal process regulation of body fluid levels wound healing response to stimulus hemostasis biological regulation blood coagulation response to stress coagulation regulation of biological quality response to wounding cellular response to stimulus positive regulation of cell communication regulation of cellular process positive regulation of signal transduction signaling positive regulation of biological process regulation of signal transduction intracellular signal transduction cell communication positive regulation of response to stimulus regulation of intracellular signal transduction positive regulation of TOR signaling signal transduction positive regulation of cellular process TOR signaling regulation of biological process regulation of signaling regulation of TOR signaling positive regulation of intracellular signal transduction regulation of response to stimulus regulation of cell communication cellular process positive regulation of signaling positive regulation of locomotion regulation of cell motility positive regulation of cell migration regulation of locomotion positive regulation of cell motility regulation of cell migration cell migration cell motility locomotion proteolysis metabolic process primary metabolic process protein metabolic process macromolecule metabolic process	cell periphery cellular anatomical structure cell surface membrane plasma membrane external side of plasma membrane side of membrane membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen cell periphery cellular anatomical structure cell surface membrane plasma membrane external side of plasma membrane side of membrane membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen Golgi lumen intracellular organelle organelle lumen organelle cytoplasm endoplasmic reticulum lumen endoplasmic reticulum extracellular region extracellular space

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR007803	Aspartyl/asparaginy/proline hydroxylase	Domain
A	IPR011990	Tetratricopeptide-like helical domain superfamily	Homologous Superfamily
A	IPR027443	Isopenicillin N synthase-like superfamily	Homologous Superfamily
A	IPR019734	Tetratricopeptide repeat	Repeat
A	IPR007943	Aspartyl beta-hydroxylase/Triadin domain	Domain
A	IPR039038	Aspartyl/asparaginyl beta-hydroxylase family	Family
B	IPR000294	Gamma-carboxyglutamic acid-rich (GLA) domain	Domain
B	IPR018097	EGF-like calcium-binding, conserved site	Conserved Site
B	IPR000742	EGF-like domain	Domain
B	IPR018114	Serine proteases, trypsin family, histidine active site	Active Site
B	IPR050442	Peptidase S1 family, coagulation factors	Family
B	IPR009003	Peptidase S1, PA clan	Homologous Superfamily
B	IPR017857	Coagulation factor-like, Gla domain superfamily	Homologous Superfamily
B	IPR001314	Peptidase S1A, chymotrypsin family	Family
B	IPR033116	Serine proteases, trypsin family, serine active site	Active Site
B	IPR001254	Serine proteases, trypsin domain	Domain
B	IPR035972	Gamma-carboxyglutamic acid-rich (GLA) domain superfamily	Homologous Superfamily
B	IPR001881	EGF-like calcium-binding domain	Domain
B	IPR043504	Peptidase S1, PA clan, chymotrypsin-like fold	Homologous Superfamily
B	IPR000152	EGF-type aspartate/asparagine hydroxylation site	PTM
B	IPR012224	Peptidase S1A, coagulation factor VII/IX/X/C/Z	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: Q12797	facial dysmorphism-lens dislocation-anterior segment abnormalities-spontaneous filtering blebs syndrome lung adenocarcinoma malignant pancreatic neoplasm osteosarcoma pancreatic intraductal papillary-mucinous carcinoma adenoma Pick disease psoriasis atypical teratoid rhabdoid tumor tuberculosis Crohn disease Duchenne muscular dystrophy glioblastoma juvenile dermatomyositis lung cancer facial dysmorphism-lens dislocation-anterior segment abnormalities-spontaneous filtering blebs syndrome lung adenocarcinoma malignant pancreatic neoplasm osteosarcoma pancreatic intraductal papillary-mucinous carcinoma adenoma Pick disease psoriasis atypical teratoid rhabdoid tumor tuberculosis Crohn disease Duchenne muscular dystrophy glioblastoma juvenile dermatomyositis lung cancer medulloblastoma ovarian cancer pancreatic intraductal papillary-mucinous neoplasm adrenal cortex carcinoma primitive neuroectodermal tumor rheumatoid arthritis breast cancer ulcerative colitis dermatomyositis ductal breast carcinoma in situ facioscapulohumeral muscular dystrophy intellectual disability large cell medulloblastoma malignant ependymoma myopathy pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia progressive supranuclear palsy atopic eczema Sjogren syndrome breast carcinoma Down syndrome Emery-Dreifuss muscular dystrophy Gaucher disease invasive ductal breast carcinoma
B	Pharos: P00742	congenital factor X deficiency atrial fibrillation factor X deficiency thrombotic disease Prevention of deep vein thrombosis pulmonary embolism blood coagulation disease pancreatic ductal adenocarcinoma lung adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm psoriasis breast fibroadenoma ductal breast carcinoma in situ invasive ductal breast carcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia

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.

Prepare Data

Validate Data

Deposit Data

Help and Resources

8RE8

Aspartyl/Asparaginyl beta-hydroxylase (AspH) R688Q variant in complex with Mn, (3R)-methyl-2-oxoglutarate and a Factor X derived peptide fragment

External Resource: Annotation

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

InterPro: Protein Family Classification InterPro Database Homepage

Pharos: Disease Associations Pharos Homepage Annotation