|
gptkbp:instanceOf
|
gptkb:protein
|
|
gptkbp:activity
|
oxidative decarboxylation
|
|
gptkbp:associatedWith
|
gptkb:maple_syrup_urine_disease
|
|
gptkbp:cofactor
|
gptkb:CoA
gptkb:FAD
NAD+
thiamine pyrophosphate
lipoic acid
|
|
gptkbp:component
|
E1 enzyme
E2 enzyme
E3 enzyme
|
|
gptkbp:deficiencyCauses
|
accumulation of branched-chain amino acids
|
|
gptkbp:discoveredBy
|
1960s
|
|
gptkbp:EC_number
|
1.2.4.4
|
|
gptkbp:fullName
|
gptkb:branched-chain_alpha-keto_acid_dehydrogenase_complex
|
|
gptkbp:function
|
catalyzes the decarboxylation of branched-chain alpha-keto acids
|
|
gptkbp:gene
|
gptkb:DBT
gptkb:DLD
gptkb:BCKDHA
gptkb:BCKDHB
|
|
https://www.w3.org/2000/01/rdf-schema#label
|
BCKD complex
|
|
gptkbp:involvedIn
|
branched-chain amino acid catabolism
|
|
gptkbp:location
|
mitochondrial matrix
|
|
gptkbp:molecularWeight
|
~4 MDa
|
|
gptkbp:OMIM
|
248600
|
|
gptkbp:orthologIn
|
mouse BCKD complex
yeast BCKD complex
|
|
gptkbp:pathway
|
amino acid degradation
|
|
gptkbp:regulates
|
phosphorylation
dephosphorylation
BCKD kinase
BCKD phosphatase
|
|
gptkbp:significance
|
inborn errors of metabolism
|
|
gptkbp:structure
|
multienzyme complex
|
|
gptkbp:substrate
|
gptkb:isoleucine
leucine
valine
|
|
gptkbp:subunit
|
gptkb:DBT
gptkb:DLD
gptkb:BCKDHA
gptkb:BCKDHB
|
|
gptkbp:UniProtID
|
P12694
P09622
P11182
P21953
|
|
gptkbp:bfsParent
|
gptkb:branched-chain_alpha-keto_acid_dehydrogenase_complex
|
|
gptkbp:bfsLayer
|
8
|