Very long chain fatty acid
Encyclopedia
A very long chain fatty acid (VLCFA) is a fatty acid
with aliphatic tails longer than 22 carbons.
Unlike most fatty acids, VLCFAs are too long to be metabolized in the mitochondria, and must be metabolized in peroxisome
s.
Certain peroxisomal disorders, such as adrenoleukodystrophy
, can be associated with an accumulation of VLCFAs.
Fatty acid
In chemistry, especially biochemistry, a fatty acid is a carboxylic acid with a long unbranched aliphatic tail , which is either saturated or unsaturated. Most naturally occurring fatty acids have a chain of an even number of carbon atoms, from 4 to 28. Fatty acids are usually derived from...
with aliphatic tails longer than 22 carbons.
Unlike most fatty acids, VLCFAs are too long to be metabolized in the mitochondria, and must be metabolized in peroxisome
Peroxisome
Peroxisomes are organelles found in virtually all eukaryotic cells. They are involved in the catabolism of very long chain fatty acids, branched chain fatty acids, D-amino acids, polyamines, and biosynthesis of plasmalogens, etherphospholipids critical for the normal function of mammalian brains...
s.
Certain peroxisomal disorders, such as adrenoleukodystrophy
Adrenoleukodystrophy
Adrenoleukodystrophy is a rare, inherited disorder that leads to progressive brain damage, failure of the adrenal glands and eventually death. ALD is a disease in a group of genetic disorders called leukodystrophies, whose chief feature is damage to myelin...
, can be associated with an accumulation of VLCFAs.
See also
- ACADVLACADVLACADVL is a gene associated with very long-chain acyl-coenzyme A dehydrogenase deficiency.-External links:*...
- SLC27A2SLC27A2Very long-chain acyl-CoA synthetase is an enzyme that in humans is encoded by the SLC27A2 gene.-Further reading:...
- SLC27A5SLC27A5Bile acyl-CoA synthetase is an enzyme that in humans is encoded by the SLC27A5 gene.-Further reading:...