Impaired membrane traffic in defective ether lipid biosynthesis

Thanh Phuong Thai, Claus Rodemer, Anna Jauch, Andreas Hunziker, Ann B. Moser, Karin Gorgas, Wilhelm W. Just

Research output: Contribution to journalArticle

Abstract

The first steps of ether lipid biosynthesis are exclusively localized to peroxisomes and hence some peroxisomal disorders are characterized by a severe deficiency of plasmalogens, the main ether lipids in humans. Here we report on gene defects of plasmalogen biosynthesis, chromosomal localization of the corresponding genes and, as a consequence of plasmalogen deficiency, on structural alterations of caveolae, clathrin-coated pits, endoplasmic reticulum and Golgi cisternae, as well as on the reduced rate of transferrin receptor cycling. The data suggest that plasmalogens, analogous to cholesterol, are essential for correct membrane functioning and their deficiency results in impaired membrane trafficking.

Original languageEnglish (US)
Pages (from-to)127-136
Number of pages10
JournalHuman Molecular Genetics
Volume10
Issue number2
StatePublished - Jan 15 2001

Fingerprint

Plasmalogens
Ether
Lipids
Membranes
Peroxisomal Disorders
Caveolae
Clathrin
Transferrin Receptors
Peroxisomes
Endoplasmic Reticulum
Genes
Cholesterol

ASJC Scopus subject areas

  • Genetics

Cite this

Thai, T. P., Rodemer, C., Jauch, A., Hunziker, A., Moser, A. B., Gorgas, K., & Just, W. W. (2001). Impaired membrane traffic in defective ether lipid biosynthesis. Human Molecular Genetics, 10(2), 127-136.

Impaired membrane traffic in defective ether lipid biosynthesis. / Thai, Thanh Phuong; Rodemer, Claus; Jauch, Anna; Hunziker, Andreas; Moser, Ann B.; Gorgas, Karin; Just, Wilhelm W.

In: Human Molecular Genetics, Vol. 10, No. 2, 15.01.2001, p. 127-136.

Research output: Contribution to journalArticle

Thai, TP, Rodemer, C, Jauch, A, Hunziker, A, Moser, AB, Gorgas, K & Just, WW 2001, 'Impaired membrane traffic in defective ether lipid biosynthesis', Human Molecular Genetics, vol. 10, no. 2, pp. 127-136.
Thai TP, Rodemer C, Jauch A, Hunziker A, Moser AB, Gorgas K et al. Impaired membrane traffic in defective ether lipid biosynthesis. Human Molecular Genetics. 2001 Jan 15;10(2):127-136.
Thai, Thanh Phuong ; Rodemer, Claus ; Jauch, Anna ; Hunziker, Andreas ; Moser, Ann B. ; Gorgas, Karin ; Just, Wilhelm W. / Impaired membrane traffic in defective ether lipid biosynthesis. In: Human Molecular Genetics. 2001 ; Vol. 10, No. 2. pp. 127-136.
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