Also known as acyl-CoA synthetase family member 3
Acyl-CoA synthetase family member 3 (ACSF3) is a mitochondrial enzyme encoded by the ACSF3 gene. It is required for the degradation of malonic acid and methylmalonic acid and provides the precursor for mitochondrial fatty acid synthesis (mtFAS) and mitochondrial lysine malonylation. The enzyme belongs to the acyl-CoA synthetase family.
This gene encodes a member of the acyl-CoA synthetase family of enzymes that activate fatty acids by catalyzing the formation of a thioester linkage between fatty acids and coenzyme A. The encoded protein is localized to mitochondria, has high specificity for malonate and methylmalonate and possesses malonyl-CoA synthetase activity. Mutations in this gene are a cause of combined malonic and methylmalonic aciduria. Alternatively spliced transcript variants have been observed for this gene. [provided by RefSeq, Sep 2013].
Biological process
~12 min read
Acyl-CoA synthetase family member 3 (ACSF3) is a mitochondrial enzyme encoded by the ACSF3 gene. It is required for the degradation of malonic acid and methylmalonic acid and provides the precursor for mitochondrial fatty acid synthesis (mtFAS) and mitochondrial lysine malonylation. The enzyme belongs to the acyl-CoA synthetase family.
== Structure == The ACSF3 gene is located on chromosome 16, at locus q24.3. It comprises 14 exons and produces four alternatively spliced mRNAs that encode two isoforms of the ACSF3 protein:
Cellular component
via MyGene.info
Discovered by embedding cosine similarity (sentence-transformers MiniLM, 384-dim).