Gene/Proteome Database (LMPD)
LMPD ID
LMP000630
Gene ID
Species
Homo sapiens (Human)
Gene Name
malonyl-CoA decarboxylase
Gene Symbol
Synonyms
MCD
Alternate Names
malonyl-CoA decarboxylase, mitochondrial; malonyl coenzyme A decarboxylase
Chromosome
16
Map Location
16q24
EC Number
4.1.1.9
Summary
The product of this gene catalyzes the breakdown of malonyl-CoA to acetyl-CoA and carbon dioxide. Malonyl-CoA is an intermediate in fatty acid biosynthesis, and also inhibits the transport of fatty acyl CoAs into mitochondria. Consequently, the encoded protein acts to increase the rate of fatty acid oxidation. It is found in mitochondria, peroxisomes, and the cytoplasm. Mutations in this gene result in malonyl-CoA decarboyxlase deficiency. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| malonyl-CoA decarboxylase, mitochondrial | |
|---|---|
| Refseq ID | NP_036345 |
| Protein GI | 110349750 |
| UniProt ID | O95822 |
| mRNA ID | NM_012213 |
| Length | 493 |
| RefSeq Status | REVIEWED |
| MRGFGPGLTARRLLPLRLPPRPPGPRLASGQAAGALERAMDELLRRAVPPTPAYELREKTPAPAEGQCADFVSFYGGLAETAQRAELLGRLARGFGVDHGQVAEQSAGVLHLRQQQREAAVLLQAEDRLRYALVPRYRGLFHHISKLDGGVRFLVQLRADLLEAQALKLVEGPDVREMNGVLKGMLSEWFSSGFLNLERVTWHSPCEVLQKISEAEAVHPVKNWMDMKRRVGPYRRCYFFSHCSTPGEPLVVLHVALTGDISSNIQAIVKEHPPSETEEKNKITAAIFYSISLTQQGLQGVELGTFLIKRVVKELQREFPHLGVFSSLSPIPGFTKWLLGLLNSQTKEHGRNELFTDSECKEISEITGGPINETLKLLLSSSEWVQSEKLVRALQTPLMRLCAWYLYGEKHRGYALNPVANFHLQNGAVLWRINWMADVSLRGITGSCGLMANYRYFLEETGPNSTSYLGSKIIKASEQVLSLVAQFQKNSKL | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005737 | IDA:UniProtKB | C | cytoplasm |
| GO:0005759 | ISS:UniProtKB | C | mitochondrial matrix |
| GO:0005739 | IDA:UniProtKB | C | mitochondrion |
| GO:0005782 | ISS:UniProtKB | C | peroxisomal matrix |
| GO:0005777 | IDA:UniProtKB | C | peroxisome |
| GO:0050080 | IDA:UniProtKB | F | malonyl-CoA decarboxylase activity |
| GO:0005102 | IPI:UniProtKB | F | receptor binding |
| GO:0006085 | IDA:UniProtKB | P | acetyl-CoA biosynthetic process |
| GO:0044255 | TAS:Reactome | P | cellular lipid metabolic process |
| GO:0006633 | IDA:UniProtKB | P | fatty acid biosynthetic process |
| GO:2001294 | IDA:UniProtKB | P | malonyl-CoA catabolic process |
| GO:0046321 | IMP:UniProtKB | P | positive regulation of fatty acid oxidation |
| GO:0010906 | IMP:UniProtKB | P | regulation of glucose metabolic process |
| GO:0002931 | ISS:UniProtKB | P | response to ischemia |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
KEGG Pathway Links
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR007956 | Malonyl-CoA decarboxylase |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative initiation; Named isoforms=2; Comment=A single transcription start site has been demonstrated in Rat.; Name=Mitochondrial; IsoId=O95822-1; Sequence=Displayed; Name=Cytoplasmic+peroxisomal; IsoId=O95822-2; Sequence=VSP_047649; Note=May be produced by alternative initiation at Met-40 of isoform mitochondrial. Alternatively, represents a proteolytic processed form of the mitochondrial form.; |
| Biophysicochemical Properties | Kinetic parameters: KM=0.36 mM for malonyl-CoA (Malonyl-CoA decarboxylase mitochondrial form), PubMed:15003260 {ECO |
| Catalytic Activity | Malonyl-CoA = acetyl-CoA + CO(2). {ECO |
| Disease | Malonyl-CoA decarboxylase deficiency (MLYCD deficiency) [MIM |
| Enzyme Regulation | Malonyl-CoA decarboxylase activity does not require any cofactors or divalent metal ions. Formation of interchain disulfide bonds leads to positive cooperativity between active sites and increases the affinity for malonyl-CoA and the catalytic efficiency (in vitro). {ECO |
| Function | Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids. Plays a role in the metabolic balance between glucose and lipid oxidation in muscle independent of alterations in insulin signaling. May play a role in controlling the extent of ischemic injury by promoting glucose oxidation. {ECO |
| Pathway | Metabolic intermediate biosynthesis; acetyl-CoA biosynthesis; acetyl-CoA from malonyl-CoA |
| Ptm | Acetylation at Lys-472 activates malonyl-CoA decarboxylase activity. Deacetylation at Lys-472 by SIRT4 represses activity, leading to promote lipogenesis (By similarity). |
| Ptm | Interchain disulfide bonds may form in peroxisomes (Potential). Interchain disulfide bonds are not expected to form in the reducing environment of the cytoplasm and mitochondria. |
| Sequence Caution | Sequence=AAD16177.2; Type=Frameshift; Positions=23, 28, 297, 308; Evidence= ; |
| Subcellular Location | Cytoplasm . Mitochondrion matrix . Peroxisome {ECO |
| Subunit | Homotetramer. Dimer of dimers. The two subunits within a dimer display conformational differences suggesting that at any given moment, only one of the two subunits is competent for malonyl-CoA binding and catalytic activity. Under oxidizing conditions, can form disulfide-linked homotetramers (in vitro). Associates with the peroxisomal targeting signal receptor PEX5. |
| Tissue Specificity | Expressed in fibroblasts and hepatoblastoma cells (at protein level). Expressed strongly in heart, liver, skeletal muscle, kidney and pancreas. Expressed in myotubes. Expressed weakly in brain, placenta, spleen, thymus, testis, ovary and small intestine. {ECO |
Identical and Related Proteins
Unique RefSeq proteins for LMP000630 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 110349750 | RefSeq | NP_036345 | 493 | malonyl-CoA decarboxylase, mitochondrial |
Identical Sequences to LMP000630 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:110349750 | GenBank | AAH52592.1 | 493 | Malonyl-CoA decarboxylase [Homo sapiens] |
| GI:110349750 | GenBank | EAW95513.1 | 493 | malonyl-CoA decarboxylase [Homo sapiens] |
| GI:110349750 | SwissProt | O95822.3 | 493 | RecName: Full=Malonyl-CoA decarboxylase, mitochondrial; Short=MCD; Flags: Precursor [Homo sapiens] |
Related Sequences to LMP000630 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:110349750 | GenBank | AAD48994.1 | 493 | malonyl coenzyme A decarboxylase [Homo sapiens] |
| GI:110349750 | GenBank | JAA02619.1 | 493 | malonyl-CoA decarboxylase [Pan troglodytes] |
| GI:110349750 | RefSeq | XP_523518.2 | 493 | PREDICTED: malonyl-CoA decarboxylase, mitochondrial [Pan troglodytes] |
| GI:110349750 | RefSeq | XP_003272531.1 | 493 | PREDICTED: malonyl-CoA decarboxylase, mitochondrial [Nomascus leucogenys] |
| GI:110349750 | RefSeq | XP_003820926.1 | 494 | PREDICTED: LOW QUALITY PROTEIN: malonyl-CoA decarboxylase, mitochondrial [Pan paniscus] |
| GI:110349750 | RefSeq | XP_004058114.1 | 493 | PREDICTED: malonyl-CoA decarboxylase, mitochondrial [Gorilla gorilla gorilla] |