Gene: [07q3/DLD] lipoamide dehydrogenase; lactoacidosis due to lipoamide dehydrogenase deficiency;
COM | Lipoamide dehydrogenase had another code, EC:1.6.4.3, until 1983. Now it corresponds to diaphorase 4 (GEM:16q221/NMOR1(DIA4))." |
GEN | Two transcripts have been found: 2.2 and 2.4 kb. cDNA contains a 3'-untranslated region with an unusual polyadenylation signal, TATAAA, and a short poly(A) sequence." |
FUN | [1] Systematic name:
dihydrolipoamide:NAD+ oxidoreductase. [2] The reaction catalyzed: dihydrolipoamide + NAD+ = lipoamide + NADH. [3] The lipoamide dehydrogenase is a component of three multi-enzyme complexes: piruvate dehydrogenase, alpha-ketoglutarate dehydrogenase and branched-chain alpha-ketoacid dehydrogenase." |
MOP | The product is a homodimer; MM of a subunit is 55 kD (474 amino acids); the protein precursor consists of 509 amino acid residues." |
REF | PRO,LOC,MOL
"Otulakowski G &: Somat Cell Mol Genet, 14, 411-414, 1988 SEQ "Otulakowski G, Robinson: JBC, 262, 17313-17318, 1987 CLO,SEQ,MOP,CAG "Pons G &: PNAS, 85, 1422-1426, 1988 PRO,LOC "Scherer SW &: CCG, 56, N3-4, 176-177, 1991 |
SWI | SWISSPROT: P09622 |
KEY | mtbd, lip, mito |
CLA | coding, basic |
LOC | 07 q31-32 |
MIM | MIM: 246900 |
EZN | ENZYME: 1.8.1.4 |
