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Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C27bile acids but normal fibroblast branched-chain fatty acid oxidation

โœ Scribed by P. T. Clayton; A. W. Johnson; K. A. Mills; G. W. Lynes; J. Wilson; M. Casteels; G. Mannaerts

Publisher
Springer
Year
1996
Tongue
English
Weight
625 KB
Volume
19
Category
Article
ISSN
0141-8955

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Phytanic acid and pristanic acid, branched-chain fatty acids associated with Refsum disease and other inherited peroxisomal disorders, mediate intracellular Ca2+ signaling through activation of free fatty acid receptor GPR40
โœ Nicol Kruska; Georg Reiser ๐Ÿ“‚ Article ๐Ÿ“… 2011 ๐Ÿ› Elsevier Science ๐ŸŒ English โš– 824 KB

The accumulation of the two branched-chain fatty acids phytanic acid and pristanic acid is known to play an important role in several diseases with peroxisomal impairment, like Refsum disease, Zellweger syndrome and ฮฑ-methylacyl-CoA racemase deficiency. Recent studies elucidated that the toxic activ