1.2.1.25: branched-chain alpha-keto acid dehydrogenase system

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For detailed information about branched-chain alpha-keto acid dehydrogenase system, go to the full flat file.

Word Map on EC 1.2.1.25

1.2.1.25

maple

syrup

pdha1

dihydrolipoamide

alpha2beta2

transacylase

keto-acids

bckdhb

dihydrolipoyl

Reaction

Synonyms

2-oxoisovalerate dehydrogenase, alpha-ketoisovalerate dehydrogenase, BCKD, BCKD complex, BCKDalpha, BCKDbeta, BCKDC, BCKDH, BCKDHA, BCKDHB, bkdA, BKDH, branched chain alpha-keto acid dehydrogenase complex, branched chain keto-acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase complex, branched-chain alpha-ketoacid dehydrogenase complex E1alpha subunit, branched-chain ketoacid dehydrogenase, branched-chain-keto-acid dehydrogenase alpha, branched-chain-keto-acid dehydrogenase beta, dehydrogenase, 2-oxoisovalerate (acylating), E1alpha subunit, E1beta subunit, mitochondrial branched-chain keto-acid dehydrogenase, More

ECTree

1 Oxidoreductases

1.2 Acting on the aldehyde or oxo group of donors

1.2.1 With NAD⁺ or NADP⁺ as acceptor

1.2.1.25 branched-chain alpha-keto acid dehydrogenase system

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Results	in table
2	Activating Compound
14	AA Sequence
1	CAS Registry Number
3	Cloned(Commentary)
30	Cofactor
114	Disease/ Diagnostics
6	Expression
35	General Information
7	Inhibitors
2	KM Value [mM]
11	Localization
38	Natural Substrates/ Products (Substrates)
188	Organism
12	Pathway
1	pH Optimum
1	pH Range
6	Posttranslational Modification
1	Protein Variants
1	Purification (Commentary)
1	Reaction
4	Reaction Type
17	Reference
56	Source Tissue
41	Substrates and Products (Substrate)
8	Subunits
47	Synonyms
1	Systematic Name

Localization

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Localization on EC 1.2.1.25 - branched-chain alpha-keto acid dehydrogenase system

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mitochondrial inner membrane

Homo sapiens

P12694; P21953

human BCKD complex is likely located on the matrix side of the inner mitochondrial membrane