Annotations: 5RVZ

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
B	OxoGdeHyase_C	e5rvzB2	A: a/b three-layered sandwiches	X: TK C-terminal domain-like (From Topology)	H: TK C-terminal domain-like (From Topology)	T: TK C-terminal domain-like	F: OxoGdeHyase_C	ECOD (1.6)
B	E1_dh	e5rvzB1	A: a/b three-layered sandwiches	X: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	H: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	T: Thiamin diphosphate-binding fold (THDP-binding)	F: E1_dh	ECOD (1.6)
B	Transket_pyr	e5rvzB3	A: a/b three-layered sandwiches	X: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	H: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	T: Thiamin diphosphate-binding fold (THDP-binding)	F: Transket_pyr	ECOD (1.6)
A	OxoGdeHyase_C	e5rvzA2	A: a/b three-layered sandwiches	X: TK C-terminal domain-like (From Topology)	H: TK C-terminal domain-like (From Topology)	T: TK C-terminal domain-like	F: OxoGdeHyase_C	ECOD (1.6)
A	E1_dh	e5rvzA1	A: a/b three-layered sandwiches	X: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	H: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	T: Thiamin diphosphate-binding fold (THDP-binding)	F: E1_dh	ECOD (1.6)
A	Transket_pyr	e5rvzA3	A: a/b three-layered sandwiches	X: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	H: Thiamin diphosphate-binding fold (THDP-binding) (From Topology)	T: Thiamin diphosphate-binding fold (THDP-binding)	F: Transket_pyr	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF02779	Transketolase, pyrimidine binding domain (Transket_pyr)	Transketolase, pyrimidine binding domain	This family includes transketolase enzymes, pyruvate dehydrogenases, and branched chain alpha-keto acid decarboxylases.	Domain
A, B	PF16870	2-oxoglutarate dehydrogenase C-terminal (OxoGdeHyase_C)	2-oxoglutarate dehydrogenase C-terminal	-	Family
A, B	PF00676	Dehydrogenase E1 component (E1_dh)	Dehydrogenase E1 component	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial	oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor oxidoreductase activity 2-oxoadipate dehydrogenase activity oxidoreductase activity, acting on the aldehyde or oxo group of donors catalytic activity oxoglutarate dehydrogenase (succinyl-transferring) activity binding cation binding vitamin binding thiamine pyrophosphate binding ion binding organic cyclic compound binding anion binding quaternary ammonium group binding oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor oxidoreductase activity 2-oxoadipate dehydrogenase activity oxidoreductase activity, acting on the aldehyde or oxo group of donors catalytic activity oxoglutarate dehydrogenase (succinyl-transferring) activity binding cation binding vitamin binding thiamine pyrophosphate binding ion binding organic cyclic compound binding anion binding quaternary ammonium group binding heterocyclic compound binding small molecule binding sulfur compound binding	ribonucleotide catabolic process pyridine nucleotide catabolic process oxoacid metabolic process carbohydrate catabolic process carbohydrate derivative metabolic process organophosphate catabolic process nucleoside phosphate catabolic process organic acid metabolic process nucleobase-containing compound metabolic process glycolytic process phosphate-containing compound metabolic process pyridine-containing compound metabolic process small molecule metabolic process purine ribonucleotide catabolic process ribonucleotide catabolic process pyridine nucleotide catabolic process oxoacid metabolic process carbohydrate catabolic process carbohydrate derivative metabolic process organophosphate catabolic process nucleoside phosphate catabolic process organic acid metabolic process nucleobase-containing compound metabolic process glycolytic process phosphate-containing compound metabolic process pyridine-containing compound metabolic process small molecule metabolic process purine ribonucleotide catabolic process phosphorus metabolic process pyridine nucleotide metabolic process nucleoside phosphate metabolic process nicotinamide nucleotide metabolic process pyruvate metabolic process purine nucleoside diphosphate metabolic process primary metabolic process purine-containing compound catabolic process nucleotide metabolic process nucleobase-containing compound catabolic process nucleoside diphosphate metabolic process purine nucleoside diphosphate catabolic process nucleoside diphosphate catabolic process metabolic process nucleoside triphosphate metabolic process monocarboxylic acid metabolic process purine ribonucleotide metabolic process pyridine-containing compound catabolic process ribose phosphate metabolic process catabolic process ATP metabolic process purine nucleotide metabolic process ribonucleotide metabolic process nucleobase-containing small molecule metabolic process purine ribonucleoside triphosphate metabolic process ribonucleoside diphosphate metabolic process ribonucleoside diphosphate catabolic process nucleotide catabolic process ribonucleoside triphosphate metabolic process purine nucleoside triphosphate metabolic process purine nucleotide catabolic process carbohydrate metabolic process carbohydrate derivative catabolic process ADP catabolic process carboxylic acid metabolic process generation of precursor metabolites and energy purine-containing compound metabolic process ADP metabolic process organophosphate metabolic process purine ribonucleoside diphosphate metabolic process cellular process purine ribonucleoside diphosphate catabolic process developmental process cellular developmental process hemopoiesis cell differentiation anatomical structure development cell development hematopoietic progenitor cell differentiation	membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle oxoadipate dehydrogenase complex protein-containing complex catalytic complex oxidoreductase complex alpha-ketoacid dehydrogenase complex membrane-enclosed lumen membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle oxoadipate dehydrogenase complex protein-containing complex catalytic complex oxidoreductase complex alpha-ketoacid dehydrogenase complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR042179	Multifunctional 2-oxoglutarate metabolism enzyme, C-terminal domain superfamily	Homologous Superfamily
A, B	IPR029061	Thiamin diphosphate-binding fold	Homologous Superfamily
A, B	IPR005475	Transketolase-like, pyrimidine-binding domain	Domain
A, B	IPR011603	2-oxoglutarate dehydrogenase E1 component	Family
A, B	IPR031717	2-oxoglutarate dehydrogenase E1 component/KDG, C-terminal	Domain
A, B	IPR001017	Dehydrogenase, E1 component	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: Q96HY7	2-aminoadipic 2-oxoadipic aciduria Charcot-Marie-Tooth disease Charcot-Marie-Tooth disease axonal type 2Q peripheral neuropathy Liver Cirrhosis, Experimental invasive ductal breast carcinoma lung carcinoma neurodegenerative disease pancreatic ductal adenocarcinoma breast cancer osteosarcoma pancreatic intraductal papillary-mucinous neoplasm subependymal giant cell astrocytoma breast carcinoma intellectual disability 2-aminoadipic 2-oxoadipic aciduria Charcot-Marie-Tooth disease Charcot-Marie-Tooth disease axonal type 2Q peripheral neuropathy Liver Cirrhosis, Experimental invasive ductal breast carcinoma lung carcinoma neurodegenerative disease pancreatic ductal adenocarcinoma breast cancer osteosarcoma pancreatic intraductal papillary-mucinous neoplasm subependymal giant cell astrocytoma breast carcinoma intellectual disability lung adenocarcinoma motor peripheral neuropathy ovarian cancer peripheral nervous system disease

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.