malonyl-CoA decarboxylase, mitochondrial; MCD (MLYCD)

Function

• fatty acid biosynthesis
• catalyzes the conversion of malonyl-CoA to acetyl-CoA
• by removing malonyl-CoA, assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase
• degradation of intraperoxisomal malonyl-CoA generated by peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids

Malonyl-CoA <--> acetyl-CoA + CO2

Compartment

mitochondria, cytoplasm, peroxisome

Alternative initiation

named isoforms=2

Pathology

• defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency

More general terms

• carboxy-lyase or decarboxylase
• mitochondrial protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=23417
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:23417
• OMIM: https://mirror.omim.org/entry/248360
• OMIM: https://mirror.omim.org/entry/606761
• UniProt: http://www.uniprot.org/uniprot/O95822.html