5I96

Crystal Structure of Human Mitochondrial Isocitrate Dehydrogenase (IDH2) R140Q Mutant Homodimer in Complex with AG-221 (Enasidenib) Inhibitor.

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

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

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
B	d5i96b_	Alpha and beta proteins (a/b)	Isocitrate/Isopropylmalate dehydrogenase-like	Isocitrate/Isopropylmalate dehydrogenase-like	Dimeric isocitrate & isopropylmalate dehydrogenases	automated matches	Human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d5i96a1	Alpha and beta proteins (a/b)	Isocitrate/Isopropylmalate dehydrogenase-like	Isocitrate/Isopropylmalate dehydrogenase-like	Dimeric isocitrate & isopropylmalate dehydrogenases	automated matches	Human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d5i96a2	Artifacts	Tags	Tags	Tags	C-terminal Tags	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)
B	SCOP2B Superfamily	Isocitrate/Isopropylmalate dehydrogenase-like	8067344	3001375	SCOP2B (2022-06-29)
A	SCOP2B Superfamily	Isocitrate/Isopropylmalate dehydrogenase-like	8067344	3001375	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)
B	Iso_dh	e5i96B2	A: a/b three-layered sandwiches	X: Isocitrate/Isopropylmalate dehydrogenase-like (From Topology)	H: Isocitrate/Isopropylmalate dehydrogenase-like (From Topology)	T: Isocitrate/Isopropylmalate dehydrogenase-like	F: Iso_dh	ECOD (1.6)
A	Iso_dh	e5i96A1	A: a/b three-layered sandwiches	X: Isocitrate/Isopropylmalate dehydrogenase-like (From Topology)	H: Isocitrate/Isopropylmalate dehydrogenase-like (From Topology)	T: Isocitrate/Isopropylmalate dehydrogenase-like	F: Iso_dh	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
B	3.40.718.10	Alpha Beta	3-Layer(aba) Sandwich	Isopropylmalate Dehydrogenase	Isopropylmalate Dehydrogenase	CATH (4.3.0)
A	3.40.718.10	Alpha Beta	3-Layer(aba) Sandwich	Isopropylmalate Dehydrogenase	Isopropylmalate Dehydrogenase	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF00180	Isocitrate/isopropylmalate dehydrogenase (Iso_dh)	Isocitrate/isopropylmalate dehydrogenase	-	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Isocitrate dehydrogenase [NADP], mitochondrial	cation binding magnesium ion binding ion binding binding metal ion binding small molecule binding isocitrate dehydrogenase [NAD(P)+] activity oxidoreductase activity oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor isocitrate dehydrogenase (NADP+) activity oxidoreductase activity, acting on CH-OH group of donors catalytic activity NAD binding nucleoside phosphate binding cation binding magnesium ion binding ion binding binding metal ion binding small molecule binding isocitrate dehydrogenase [NAD(P)+] activity oxidoreductase activity oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor isocitrate dehydrogenase (NADP+) activity oxidoreductase activity, acting on CH-OH group of donors catalytic activity NAD binding nucleoside phosphate binding purine nucleotide binding organic cyclic compound binding adenyl nucleotide binding nucleotide binding heterocyclic compound binding	aerobic respiration cellular respiration tricarboxylic acid cycle energy derivation by oxidation of organic compounds metabolic process cellular process primary metabolic process generation of precursor metabolites and energy carbohydrate metabolic process glyoxylate metabolic process glyoxylate cycle oxoacid metabolic process carboxylic acid metabolic process organic acid metabolic process aerobic respiration cellular respiration tricarboxylic acid cycle energy derivation by oxidation of organic compounds metabolic process cellular process primary metabolic process generation of precursor metabolites and energy carbohydrate metabolic process glyoxylate metabolic process glyoxylate cycle oxoacid metabolic process carboxylic acid metabolic process organic acid metabolic process aldehyde metabolic process small molecule metabolic process monocarboxylic acid metabolic process isocitrate metabolic process tricarboxylic acid metabolic process organic hydroxy compound metabolic process alcohol metabolic process secondary alcohol metabolic process 2-oxoglutarate metabolic process dicarboxylic acid metabolic process nucleoside phosphate metabolic process nicotinamide nucleotide metabolic process nucleotide metabolic process purine-containing compound metabolic process NADP metabolic process nucleobase-containing compound metabolic process purine nucleotide metabolic process organophosphate metabolic process nucleobase-containing small molecule metabolic process phosphate-containing compound metabolic process pyridine-containing compound metabolic process phosphorus metabolic process pyridine nucleotide metabolic process	membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle peroxisome microbody cytosol membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR004790	Isocitrate dehydrogenase NADP-dependent	Family
A, B	IPR024084	Isopropylmalate dehydrogenase-like domain	Domain
A, B	IPR019818	Isocitrate/isopropylmalate dehydrogenase, conserved site	Conserved Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: P48735	Ollier disease cholangiocarcinoma 2-hydroxyglutaric aciduria chondroma Maffucci syndrome acute myeloid leukemia oligodendroglioma hemangioma oligoastrocytoma d-2-hydroxyglutaric aciduria 2 gemistocytic astrocytoma Neoplasm Recurrence, Local visual epilepsy osteoporosis acute promyelocytic leukemia Ollier disease cholangiocarcinoma 2-hydroxyglutaric aciduria chondroma Maffucci syndrome acute myeloid leukemia oligodendroglioma hemangioma oligoastrocytoma d-2-hydroxyglutaric aciduria 2 gemistocytic astrocytoma Neoplasm Recurrence, Local visual epilepsy osteoporosis acute promyelocytic leukemia intramuscular hemangioma kidney failure anaplastic oligodendroglioma placental hemangioma primary myelofibrosis fibrillary astrocytoma central nervous system disease hydronephrosis adult oligodendroglioma anaplastic astrocytoma angioimmunoblastic T-cell lymphoma protoplasmic astrocytoma mature T-cell and NK-cell non-Hodgkin lymphoma epithelioid hemangioma childhood oligodendroglioma hypertrophic cardiomyopathy intrahepatic cholangiocarcinoma anaplastic oligoastrocytoma bone disease breast carcinoma genetic endocrine growth disease subependymoma tuberculosis osteoarthritis hematopoietic and lymphoid system neoplasm inborn errors of metabolism osteosarcoma D-2-hydroxyglutaric aciduria alopecia pancreatic intraductal papillary-mucinous neoplasm dilated cardiomyopathy lung adenocarcinoma astrocytoma (excluding glioblastoma) ependymoma malignant ependymoma bile duct cancer myelofibrosis brain disease cancer glioblastoma glioma susceptibility 1 osteoarthritis susceptibility 1 congenital anemia intellectual disability ovarian cancer D-2-hydroxyglutaric aciduria 1 invasive ductal breast carcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia L-2-hydroxyglutaric aciduria plasma cell myeloma lung cancer biliary tract neoplasm gastric carcinoma breast cancer non-small cell lung carcinoma spinal cord disease glioma hematologic disease acne aleukemic leukemia pancreatic ductal adenocarcinoma ductal breast carcinoma in situ large cell medulloblastoma atypical teratoid rhabdoid tumor psoriasis

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.