GENE REFERENCE / HGNC:7150

MLYCD

malonyl-CoA decarboxylase

protein-coding gene16q23.3Gene information

What does this gene do?

The MLYCD gene provides instructions for making an enzyme called malonyl-CoA decarboxylase. This enzyme helps regulate the formation and breakdown of a group of fats called fatty acids. Many tissues, including heart (cardiac) muscle, use fatty acids as a major source of energy. The body also uses fatty acids to build cell membranes, produce hormones, and carry out many other important processes.

Malonyl-CoA decarboxylase is responsible for the chemical reaction that converts a molecule called malonyl-CoA to a molecule called acetyl-CoA. This reaction is an important step in the breakdown of fatty acids. Acetyl-CoA is then used to make new fatty acids and can also be used to produce energy.

Malonyl-CoA decarboxylase is most active in cardiac muscle and in muscles used for movement (skeletal muscles). It is also found in other organs and tissues, including the brain, small intestine, liver, kidney, and pancreas. This enzyme probably functions in several parts of the cell, including mitochondria, which are cells' energy-producing centers, and peroxisomes, which are small sacs that process fatty acids and other molecules. Malonyl-CoA decarboxylase also functions in the fluid that surrounds these cell structures (the cytoplasm).

Source: MedlinePlus, National Library of Medicine. Source publication: 2023-07-26. Source review: 2010-01.

ClinGen reviews

ClinGen reviews the evidence linking a particular gene to a condition. Each rating below describes the strength of that link. It does not classify a variant in your DNA file.

Condition	Inheritance	Evidence
malonic aciduriaMONDO:0009556 · Evaluated 2018-06-26	Autosomal recessive	DefinitiveFatty Acid Oxidation Disorders Gene Curation Expert Panel

ClinVar records

These numbers show what has been submitted to ClinVar. They cannot tell you how likely you are to have a condition.

Submitted alleles: 851
Submissions for variants in or overlapping this gene: 1,151
Submissions explicitly reporting this gene: 1,057
Alleles reported pathogenic / likely pathogenic: 78
VariationIDs with uncertain significance: 296
VariationIDs with conflicting classifications: 24

The rows count different things: alleles, submissions or VariationIDs. Their categories can overlap, so do not add them together. Pathogenic/likely pathogenic counts exclude structural variants that merely overlap a gene. A dash in the source means “not reported,” not zero.

NCBI produces the gene-summary file each week using counts recorded at the start of the month. The numbers may differ from those on the live ClinVar website. Read the field definitions ↗

View this gene in ClinVar ↗

YOUR DNA FILE

Can your DNA file tell you about MLYCD?

From a DNA test kit (such as 23andMe, AncestryDNA or MyHeritage). These kits check a fixed list of about 600,000 to 700,000 positions across your whole genome. ClinVar lists 78 alleles in MLYCD reported as pathogenic or likely pathogenic. A kit checks only some of these positions, and often none of them.

From whole genome sequencing (a VCF file). Sequencing reads most of your genome. Your file lists the places where your DNA differs from the reference genome. Some regions are hard to sequence, including parts of some genes that have near-identical copies elsewhere, and a VCF file does not record which positions were read well.

If a variant is not in your file, that does not mean you don’t have it.

What BioDecode checks

BioDecode compares every variant in your file with ClinVar, on your own computer. For each match, it shows the ClinVar classification, the review status and the condition it was reported for. Your file is not uploaded.

A ClinVar classification applies to one particular variant and the condition it was reported for. It does not tell you about other variants in MLYCD.

BioDecode cannot fill gaps in your test, diagnose a condition or replace clinical testing.

Buy BioDecode See a sample report →Check file compatibility →