branched-chain alpha-keto acid dehydrogenase complex

GPTKB entity

Predicate	Object
gptkbp:instanceOf	gptkb:enzyme
gptkbp:alsoKnownAs	gptkb:BCKD_complex
gptkbp:associatedWith	gptkb:DBT gptkb:DLD gptkb:BCKDHA gptkb:BCKDHB
gptkbp:catalyzes	oxidative decarboxylation of branched-chain alpha-keto acids
gptkbp:cofactor	gptkb:Coenzyme_A gptkb:FAD NAD+ thiamine pyrophosphate lipoic acid
gptkbp:composedOf	gptkb:E1_component gptkb:E2_component gptkb:E3_component
gptkbp:deficiencyCauses	gptkb:maple_syrup_urine_disease
gptkbp:EC_number	1.2.4.4
gptkbp:foundIn	humans mammals
https://www.w3.org/2000/01/rdf-schema#label	branched-chain alpha-keto acid dehydrogenase complex
gptkbp:involvedIn	branched-chain amino acid catabolism
gptkbp:locatedIn	mitochondria
gptkbp:molecularWeight	~4,000 kDa
gptkbp:product	gptkb:alpha-methylbutyryl-CoA gptkb:isobutyryl-CoA gptkb:isovaleryl-CoA
gptkbp:regulates	phosphorylation dephosphorylation
gptkbp:substrate	gptkb:alpha-keto-beta-methylvalerate gptkb:alpha-ketoisocaproate gptkb:alpha-ketoisovalerate
gptkbp:bfsParent	gptkb:BCKDHA
gptkbp:bfsLayer	7