Resistance to erucic acid as a selectable marker for peroxisomal activity: isolation of revertants of an infantile Refsum disease cell line.

doi:10.1007/BF00735394

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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...

Topics

Acyltransferases
Albumins
Autoradiography
Biomarkers
Cell Line
DNA
Erucic Acids
Fibroblasts
Fluorescent Antibody Technique
Humans
Immunoblotting

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Immunohistochemistry

Infant, Newborn

Lipids

Microbodies

Mutation

Refsum Disease

Transfection

Source

Open in PubMed

Identifiers

PMID: 7519689
DOI: 10.1007/BF00735394

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-7519689-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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