Gene
MLYCD
malonyl-CoA decarboxylase
- HGNC 7150(opens in a new tab)
- Ensembl gene ENSG00000103150(opens in a new tab)
- NCBI Gene 23417(opens in a new tab)
Summary
- Cytogenetic location
- 16q23.3
- Coordinates
- Chromosome 16: 83,899,115–83,927,031 (GRCh38)
- Exons
- 5 exons across 27.9 kb
- Protein
- 493 amino acids
- Inheritance
- Autosomal recessive
- Also known as
- MCD, hMCD
- MANE Select transcript
- ENST00000262430.6NM_012213.3
- Locus type
- gene with protein product
- Very high
- Confident
- Low
- Very low
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Classic Organic Acidurias
- Malonic AciduriaAutosomal recessive
Caused by variants in MLYCD, which can cause developmental delay, low blood sugar, heart muscle disease and metabolic acidosis.
- ClinGen rates the link definitive
- 4 GenCC submitters rate it definitive or strong
What knowing changes
Knowing allows a low-fat, high-carbohydrate diet, carnitine and heart monitoring.
Inheritance
- Autosomal recessive
- It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.
How MLYCD is laid out
- Coding sequence
- Untranslated region
- Intron
- Direction of transcription
- Known disease-causing sites
- Stretches short reads find hard
Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.
Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).
Nearby genes from Ensembl release 116, on GRCh38.
Exons make up 13.1 kb of the gene's 27.9 kb; 1,482 of those bases code for the protein.
How a genome test reads MLYCD
57
positions in MLYCD where ClinVar classes a change as pathogenic or likely pathogenic
None of them falls in a region short reads find hard.
A whole genome covers all 27.9 kb of MLYCD, introns included, not only the 13.1 kb of exons.
Traits linked to MLYCD
The Human Phenotype Ontology lists these as features of the rare conditions MLYCD can cause. Each trait's page lists the other genes linked to it.
Laboratory abnormality
- Reduced malonyl-CoA decarboxylase activity in cultured fibroblasts
- Elevated urine malonic acid level
- Methylmalonic aciduria
- Low blood sugar
- Metabolic acidosis
- High levels of ketone bodies
- Elevated circulating malonyl carnitine concentration
- Elevated urine suberic acid level
Show 1 more trait
External links
Gene records
Transcript
Protein
Where this comes from
- HGNC: the gene's symbol, name, other symbols and location.
- MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
- GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
- The ACMG secondary findings list, SF v3.2.
- ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
- The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
Cite this page
Dante Labs. (n.d.). MLYCD gene: malonyl-CoA decarboxylase. Retrieved 16 October 2026, from https://dantelabs.com/genes/mlycd
Dante Labs. MLYCD gene: malonyl-CoA decarboxylase [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/mlycd
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