Article
Resistance to erucic acid as a selectable marker for peroxisomal activity: isolation of revertants of an infantile Refsum disease cell line.
Journal of inherited metabolic disease - 1 Jan 1994
Bachir Bioukar E, Straehli F, Ng K H, Rolland M O, Hashimoto T, Carreau J P, Deschatrette J
Abstract excerpt
A system based on the ability of cells to oxidize very long-chain fatty acids (VLCFA) was developed to select in vitro normal human fibroblasts from fibroblasts of patients suffering from peroxisomal disorders with multienzymatic deficiencies: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease (IRD). Cells treated with various concentrations of erucic acid (C22:1 n-9) revealed an enhanced...
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