Showing metabocard for Lipoic acid (BMDB0001451)

General function:

Involved in dihydrolipoyllysine-residue acetyltransfera

Specific function:

The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Gene Name:

DLAT

Uniprot ID:

P11180

Molecular weight:

1066.0

General function:

Lipid transport and metabolism

Specific function:

Catalyzes the activation of fatty acids by CoA to produce an acyl-CoA, the first step in fatty acid metabolism (PubMed:11382754, PubMed:10561077). Capable of activating medium-chain fatty acids (e.g. butyric (C4) to decanoic (C10) acids), and certain carboxylate-containing xenobiotics, e.g. benzoate (PubMed:10561077, PubMed:11382754). Also catalyzes the activation of lipoate to lipoyl-nucleoside monophosphate (PubMed:11382754). Activates lipoate with GTP at a 1000-fold higher rate than with ATP and activates both (R)- and (S)-lipoate to the respective lipoyl-GMP, with a preference for (R)-lipoate (PubMed:11382754).

Gene Name:

ACSM1

Uniprot ID:

Q9BEA2

Molecular weight:

64923.0

General function:

Energy production and conversion

Specific function:

Dihydrolipoamide succinyltransferase (E2) component of the 2-oxoglutarate dehydrogenase complex (By similarity). The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2) (By similarity). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A (By similarity).

Gene Name:

DLST

Uniprot ID:

P11179

Molecular weight:

48973.0

General function:

Energy production and conversion

Specific function:

Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.

Gene Name:

PDHX

Uniprot ID:

P22439

Molecular weight:

53886.0

General function:

Energy production and conversion

Specific function:

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.

Gene Name:

DBT

Uniprot ID:

P11181

Molecular weight:

53410.0

General function:

Amino acid transport and metabolism

Specific function:

The glycine cleavage system catalyzes the degradation of glycine.

Gene Name:

AMT

Uniprot ID:

P25285

Molecular weight:

42869.0

General function:

Amino acid transport and metabolism

Specific function:

The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST).

Gene Name:

GCSH

Uniprot ID:

P20821

Molecular weight:

18791.0

General function:

Not Available

Specific function:

Not Available

Gene Name:

DLD

Uniprot ID:

F1N206

Molecular weight:

54187.0

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