phytanic acid การใช้

• This results in the over-accumulation of very long chain fatty acids and branched chain fatty acids, such as phytanic acid.
• He showed, for example, that in cows fed grass silage the phytanic acid found in their plasma lipids originates from chlorophyll.
• RCDP1 patients can develop large tissue stores of branched chain fatty acids, such as phytanic acid, and show reduced levels of plasmalogens.
• It can be treated by dietary elimination of pristanic and phytanic acid through reduced intake of dairy products and meats such as beef, lamb, and chicken.
• In ruminants, the gut fermentation of ingested plant materials liberates phytol, a constituent of chlorophyll, which is then converted to phytanic acid and stored in fats.
• This mutation on the PEX7 gene is also on chromosome 6q22-24, and was found in patients presenting with accumulation of phytanic acid with no PHYH mutation.
• Insects, such as the sumac flea beetle, are reported to use phytol and its metabolites ( e . g . phytanic acid ) as chemical deterrents against predation.
• 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.
• This deficiency falls within a class of disorders called peroxisome biogenesis disorders ( PBDs ), although it is quite different from other peroxisomal disorders and does not share classic DHCA and EHCA and to a lesser extent Very long chain fatty acid and phytanic acid.
• In mammalian cells, the enzyme is responsible for converting ( 2 " R " )-methylacyl-CoA esters to their ( 2 " S " )-methylacyl-CoA epimers and known substrates, including coenzyme A esters of pristanic acid ( mostly derived from phytanic acid, a 3-methyl branched-chain fatty acid that is abundant in the diet ) and bile acids derived from cholesterol.
• Thirteen other syndromes may exhibit signs similar to Usher syndrome, including Alport syndrome, Alstrom syndrome, Bardet-Biedl syndrome, Cockayne syndrome, spondyloepiphyseal dysplasia congenita, Flynn-Aird syndrome, Friedreich ataxia, Hurler syndrome ( MPS-1 ), Kearns-Sayre syndrome ( CPEO ), Norrie syndrome, osteopetrosis ( Albers-Schonberg disease ), Refsum's disease ( phytanic acid storage disease ), and Zellweger syndrome ( cerebrohepatorenal syndrome ).