Medical Subject Headings

Last uploaded: August 28, 2024

Preferred Name	3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Synonyms	Alpha-Keto Acid Dehydrogenase Acid Dehydrogenase, Alpha-Keto Oxo-Acid Dehydrogenase, Branched-Chain Branched-Chain 2-Oxo Acid Dehydrogenase Dehydrogenase, Branched-Chain Oxo-Acid Decarboxylase, BCKA Branched-Chain Keto Acid Dehydrogenase 2-oxoisocaproate dehydrogenase 2-Oxoisocaproate Dehydrogenase Branched Chain Oxo Acid Dehydrogenase Branched Chain Ketoacid Dehydrogenase Dehydrogenase, Alpha-Keto Acid Branched-Chain Oxo-Acid Dehydrogenase Branched Chain Keto Acid Dehydrogenase Branched Chain Alpha-Keto Acid Decarboxylase 2-Oxoisovalerate Dehydrogenase (Lipoamide) 2 Oxoisocaproate Dehydrogenase Dehydrogenase, 2-Oxoisocaproate Branched Chain Alpha Keto Acid Decarboxylase Branched Chain 2 Oxo Acid Dehydrogenase BCKA Decarboxylase Alpha Keto Acid Dehydrogenase
Definitions	A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
ID	http://purl.bioontology.org/ontology/MESH/D042942
altLabel	Alpha-Keto Acid Dehydrogenase Acid Dehydrogenase, Alpha-Keto Oxo-Acid Dehydrogenase, Branched-Chain Branched-Chain 2-Oxo Acid Dehydrogenase Dehydrogenase, Branched-Chain Oxo-Acid Decarboxylase, BCKA Branched-Chain Keto Acid Dehydrogenase 2-oxoisocaproate dehydrogenase 2-Oxoisocaproate Dehydrogenase Branched Chain Oxo Acid Dehydrogenase Branched Chain Ketoacid Dehydrogenase Dehydrogenase, Alpha-Keto Acid Branched-Chain Oxo-Acid Dehydrogenase Branched Chain Keto Acid Dehydrogenase Branched Chain Alpha-Keto Acid Decarboxylase 2-Oxoisovalerate Dehydrogenase (Lipoamide) 2 Oxoisocaproate Dehydrogenase Dehydrogenase, 2-Oxoisocaproate Branched Chain Alpha Keto Acid Decarboxylase Branched Chain 2 Oxo Acid Dehydrogenase BCKA Decarboxylase Alpha Keto Acid Dehydrogenase
AQL	AD AE AI AN BI BL CF CH CL CS DE DF EC GE HI IM IP ME PD PH PK PO RE SD ST TO TU UL UR
cui	C0046494
DC	1
definition	A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
DX	20040101
FX	D008375
HN	2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003
Inverse of AQ	http://purl.bioontology.org/ontology/MESH/Q000097 http://purl.bioontology.org/ontology/MESH/Q000096 http://purl.bioontology.org/ontology/MESH/Q000172 http://purl.bioontology.org/ontology/MESH/Q000627 http://purl.bioontology.org/ontology/MESH/Q000648 http://purl.bioontology.org/ontology/MESH/Q000493 http://purl.bioontology.org/ontology/MESH/Q000494 http://purl.bioontology.org/ontology/MESH/Q000276 http://purl.bioontology.org/ontology/MESH/Q000138 http://purl.bioontology.org/ontology/MESH/Q000134 http://purl.bioontology.org/ontology/MESH/Q000266 http://purl.bioontology.org/ontology/MESH/Q000378 http://purl.bioontology.org/ontology/MESH/Q000008 http://purl.bioontology.org/ontology/MESH/Q000009 http://purl.bioontology.org/ontology/MESH/Q000032 http://purl.bioontology.org/ontology/MESH/Q000037 http://purl.bioontology.org/ontology/MESH/Q000191 http://purl.bioontology.org/ontology/MESH/Q000302 http://purl.bioontology.org/ontology/MESH/Q000145 http://purl.bioontology.org/ontology/MESH/Q000592 http://purl.bioontology.org/ontology/MESH/Q000737 http://purl.bioontology.org/ontology/MESH/Q000502 http://purl.bioontology.org/ontology/MESH/Q000506 http://purl.bioontology.org/ontology/MESH/Q000187 http://purl.bioontology.org/ontology/MESH/Q000235 http://purl.bioontology.org/ontology/MESH/Q000633 http://purl.bioontology.org/ontology/MESH/Q000600 http://purl.bioontology.org/ontology/MESH/Q000652 http://purl.bioontology.org/ontology/MESH/Q000528
Inverse of RB	EC 1.2.4.4
Inverse of RO	http://purl.bioontology.org/ontology/MESH/D008375
Machine permutation	2004; 2-OXOISOVALERATE DEHYDROGENASE (LIPOAMIDE) (now 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE)) was indexed under KETONE OXIDOREDUCTASES & MULTIENZYME COMPLEXES 1982-2003, under AMINO ACIDS, BRANCH-CHAIN 1979-1981, under KETO ACIDS 1973-1981, under CARBOXY-LYASES 1974-1979
MDA	20030709
MMR	20130709
MN	D08.811.682.657.350.760
notation	D042942
prefLabel	3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
RR	9082-72-8 (CRN)
TERMUI	T107440 T107441 T107433 T526353 T107432 T107443 T107437 T107445 T107438 T107444
TH	NLM (2004)
tui	T116 T126
subClassOf	http://purl.bioontology.org/ontology/MESH/D007658

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Mapping To	Ontology	Source
http://purl.bioontology.org/ontology/SNOMEDCT/77322005	SNOMEDCT	CUI
http://purl.bioontology.org/ontology/NDFRT/N0000167916	NDFRT	CUI
http://purl.bioontology.org/ontology/SCTSPA/77322005	SCTSPA	CUI
http://purl.bioontology.org/ontology/SNMI/F-66A60	SNMI	CUI
http://purl.bioontology.org/ontology/MSHFRE/D042942	MSHFRE	CUI
http://purl.bioontology.org/ontology/CSP/4000-0114	CRISP	CUI
http://id.nlm.nih.gov/mesh/D042942	MDM	LOOM
http://purl.obolibrary.org/obo/MESH_D042942	BERO	LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#D042942	RH-MESH	LOOM
http://www.phoc.org.cn/pmo/class/PMO_00020321	PMAPP-PMO	LOOM
http://purl.jp/bio/4/id/200906082084146040	IOBC	LOOM
http://purl.obolibrary.org/obo/OMIT_0023200	OMIT	LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#D08.811.682.657.350.760	RH-MESH	LOOM