Ads
related to: very long chain fatty acid- LC-FAOD Types
View Information About Different
Types Of LC-FAOD. Learn More.
- Treatment Available
DOJOLVI Is Available For Use.
Explore Treatment For Patients.
- Download HCP Resources
Visit The HCP Website To Download
HCP Materials. Learn More Here.
- Dosing Information
View Dosing Information About
An LC-FAOD Treatment Option.
- Sign Up For Updates Today
Stay Informed. Sign Up For Updates.
An FDA-Approved Treatment.
- Watch Preparation Videos
Learn How To Measure And Mix
This LC-FAOD Treatment Option.
- LC-FAOD Types
Search results
Results From The WOW.Com Content Network
A very-long-chain fatty acid (VLCFA) is a fatty acid with 22 or more carbons. Their biosynthesis occurs in the endoplasmic reticulum. [1] VLCFA's can represent up to a few percent of the total fatty acid content of a cell.
Very long-chain acyl-coenzyme A dehydrogenase deficiency is a fatty-acid metabolism disorder which prevents the body from converting certain fats to energy, particularly during periods without food. [1] [2] [3] Those affected by this disorder have inadequate levels of an enzyme that breaks down a group of fats called very long-chain fatty acids.
Very long-chain acyl-CoA synthetase is an enzyme that in humans is encoded by the SLC27A2 gene. [ 5 ] [ 6 ] The protein encoded by this gene is an isozyme of long-chain fatty-acid-coenzyme A ligase family.
Short-chain fatty acids (SCFAs) are fatty acids with aliphatic tails of five or fewer carbons (e.g. butyric acid). [7] Medium-chain fatty acids (MCFAs) are fatty acids with aliphatic tails of 6 to 12 [8] carbons, which can form medium-chain triglycerides. Long-chain fatty acids (LCFAs) are fatty acids with aliphatic tails of 13 to 21 carbons. [9]
It is a result of fatty acid buildup caused by failure of peroxisomal fatty acid beta oxidation which results in the accumulation of very long chain fatty acids in tissues throughout the body. The most severely affected tissues are the myelin in the central nervous system , the adrenal cortex , and the Leydig cells in the testes.
The same enzymes are used in peroxisomes as in the mitochondrial matrix and acetyl-CoA is generated. Very long chain (greater than C-22) fatty acids, branched fatty acids, [9] some prostaglandins and leukotrienes [10] undergo initial oxidation in peroxisomes until octanoyl-CoA is formed, at which point it undergoes mitochondrial oxidation. [11]
Ad
related to: very long chain fatty acid