Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase

Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase

Refsum disease is a peroxisomal disorder characterized by adult-onset retinitis pigmentosa, anosmia, sensory neuropathy, ataxia, and an accumulation of phytanic acid in plasma and tissues. Approximately 45% of cases are caused by mutations in phytanoyl-CoA hydroxylase (PAHX), the enzyme catalyzing t...

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Bibliographic Details
Main Authors: Veerle Foulon, Stanny Asselberghs, Wendy Geens, Guy P. Mannaerts, Minne Casteels, Paul P. Van Veldhoven
Format: Article
Language:English
Published: Elsevier 2003-12-01
Series:Journal of Lipid Research
Subjects:
α-oxidation
3-methyl-branched fatty acids
phytanic acid
peroxisomes
dioxygenases
stereochemistry
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520319532

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