Acetyl-CoA carboxylase 1

Gene:

ACACA Enzyme: EC 6.4.1.2 This enzyme has known catalytic activity involved in reaction: hydrogencarbonate + acetyl-CoA + ATP = malonyl-CoA + ADP + phosphate + H(+) Disease Acetyl-CoA carboxylase-alpha deficiency: An autosomal recessive inborn error of de novo fatty acid synthesis associated with severe brain damage, persistent myopathy and poor growth.

Organism:

Homo sapiens (Human)

Synonyms:

ACAC, ACC1, ACCA

Uniprot:

Q13085go to UniProt

Biological function:

Cytosolic enzyme that catalyzes the carboxylation of acetyl-CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis (PubMed:20457939, PubMed:20952656, PubMed:29899443). This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of ... [show more]go to UniProt

Representative structure for UniProt Q13085

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PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)
PDB 8xl2 chain D (observed residues)

PDB chain shown: 8xl2 D go to PDBe

UniProt residues 1 - 2346

Coverage: 56%

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10
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341
Similarity

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80
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