In STGD4, a butterfly pattern of dystrophy is caused by mutations in a gene that encodes a membrane bound protein that is involved in the elongation of very long chain fatty acids ( ELOVL4)
32.
They are involved in the catabolism of very long chain fatty acids, D-amino acids, polyamines, and biosynthesis of plasmalogens, etherphospholipids critical for the normal function of mammalian brains and lungs.
33.
The crop is grown for its seed oil, which contains long chain fatty acids ( greater than 20 carbons ), has high oxidative stability, does not have any odor and is highly emollient.
34.
In collaboration with Samuel Gurin at the University of Pennsylvania, Brady discovered the enzyme system for the biosynthesis of long chain fatty acids, and later discovered the role of malonate coenzyme A in this process.
35.
As a result of impaired peroxisome function, an individual's tissues and cells can accumulate very long chain fatty acids ( VLCFA ) and branched chain fatty acids ( BCFA ) that are normally degraded in peroxisomes.
36.
Cerotic acid is also a type of very long chain fatty acid that is often associated with the disease adrenoleukodystrophy, which involves the excessive saturation of unmetabolized fatty acid chains, including cerotic acid, in the peroxisome.
37.
The increased levels of malonyl-CoA caused by hyperglycemia and hyperinsulinemia inhibit CPT1, which causes a subsequent decrease in the transport of long chain fatty acids into muscle and heart mitochondria, decreasing fatty acid oxidation in such cells.
38.
Carnitine is needed to transport long chain fatty acids into the mitochondria, where they can be broken down to produce acetyl-CoA . Individuals with SPCD cannot produce ketone bodies as energy due to the interruption of fatty acid oxidation.
39.
Adult Refsum disease should not be confused with infantile Refsum disease, a peroxisome biogenesis disorder resulting from deficiencies in the catabolism of very long chain fatty acids and branched chain fatty acids ( such as phytanic acid ) and plasmalogen biosynthesis.
40.
This hepoxilin is esterified to sphinganine in a lipid complex termed EOS ( i . e . esterified omega-hydroxyacyl-sphingosine, see Lipoxygenase # Biological function and classification # Human lipoxygenases ) that also contains a very long chain fatty acid.
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