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Information on EC 1.2.4.4 - 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) and Organism(s) Danio rerio

for references in articles please use BRENDA:EC1.2.4.4
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IUBMB Comments
Contains thiamine diphosphate. It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
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Danio rerio
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Word Map
The taxonomic range for the selected organisms is: Danio rerio
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
bckdh, bckdhb, bckdha, branched-chain 2-oxo acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase, alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase, bckdc, branched-chain alpha-ketoacid dehydrogenase complex, bcka dehydrogenase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
2-oxoisocaproate dehydrogenase
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2-oxoisovalerate dehydrogenase (lipoate)
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alpha-keto-beta-methylvalerate dehydrogenase
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alpha-ketoisocaproate dehydrogenase
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alpha-ketoisocaproic dehydrogenase
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alpha-ketoisocaproic-alpha-keto-beta-methylvaleric dehydrogenase
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alpha-ketoisovalerate dehydrogenase
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alpha-oxoisocaproate dehydrogenase
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BCDH
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BCKADC
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BCKDH
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BCKDH E1-alpha
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BCKDH E1-beta
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BCOAD
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branched chain keto acid dehydrogenase
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branched-chain (-2-oxoacid BCD) dehydrogenase
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branched-chain 2-keto acid dehydrogenase
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branched-chain 2-oxo acid dehydrogenase
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branched-chain alpha-keto acid dehydrogenase
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branched-chain alpha-oxo acid dehydrogenase
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branched-chain keto acid dehydrogenase
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branched-chain ketoacid dehydrogenase
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dehydrogenase, 2-oxoisocaproate
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dehydrogenase, 2-oxoisovalerate (lipoate)
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dehydrogenase, branched chain alpha-keto acid
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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oxidative decarboxylation
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redox reaction
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reduction
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SYSTEMATIC NAME
IUBMB Comments
3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating)
Contains thiamine diphosphate. It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
CAS REGISTRY NUMBER
COMMENTARY hide
9031-05-4
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9082-72-8
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
A1L2C0_DANRE
391
0
43028
TrEMBL
Mitochondrion (Reliability: 1)
Q4VBU0_DANRE
446
0
50779
TrEMBL
Mitochondrion (Reliability: 2)