Sequence in raw or FASTA format:
ACSF3 acyl-CoA synthetase family member 3 [Homo sapiens (human)]
|Entrez Gene ID||197322|
|Full Name||acyl-CoA synthetase family member 3|
|General protein information|
|Organism||Homo sapiens (human)|
|Summary||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].|
|Homo sapiens (human)||ACSF3||NP_777577.2|
|Pan troglodytes (chimpanzee)||ACSF3||XP_511166.3|
|Canis lupus familiaris (dog)||ACSF3||XP_005620620.1|
|Bos taurus (cattle)||ACSF3||NP_001030240.1|
|Mus musculus (house mouse)||Acsf3||NP_659181.2|
|Rattus norvegicus (Norway rat)||Acsf3||XP_574249.3|
|Gallus gallus (chicken)||ACSF3||XP_004944364.1|
|Danio rerio (zebrafish)||LOC562292||XP_690782.2|
|Arabidopsis thaliana (thale cress)||AT3G16170||NP_566537.1|
|Xenopus (Silurana) tropicalis (western clawed frog)||acsf3||NP_001121382.2|
|GO:0006631||fatty acid metabolic process||IDA|
|GO:0006633||fatty acid biosynthetic process||IDA|
|GO:0090410||malonate catabolic process||IDA|
|GO:0016878||acid-thiol ligase activity||IDA|
|GO:0090409||malonyl-CoA synthetase activity||IDA|
What is the normal function of the ACSF3 gene?
The ACSF3 gene provides instructions for making an enzyme involved in the formation (synthesis) of fatty acids, which are building blocks used to make fats (lipids). The ACSF3 enzyme performs a chemical reaction that converts malonic acid to malonyl-CoA, which is the first step of fatty acid synthesis. Based on this activity, the enzyme is classified as a malonyl-CoA synthetase. The ACSF3 enzyme also converts methylmalonic acid to methylmalonyl-CoA, making it a methylmalonyl-CoA synthetase as well.
Fatty acid synthesis occurs through two pathways, one of which takes place in cellular structures called mitochondria. Mitochondria convert the energy from food into a form that cells can use, and fatty acid synthesis in these structures is thought to be important for their proper functioning. The ACSF3 enzyme is found only in mitochondria and is involved in mitochondrial fatty acid synthesis.
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