Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins

Lelita T. Braiterman, Siqun Zheng, Paul A. Watkins, Michael T. Geraghty, Gerald Johnson, Martina C. McGuinness, Ann B. Moser, Kirby D. Smith

Research output: Contribution to journalArticlepeer-review

Abstract

X-Linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by reduced peroxisomal very long chain fatty acid (VLCFA) β-oxidation. The X-ALD gene product (ALDP) is a peroxisomal transmembrane protein with an ATP binding cassette (ABC). ALDP and three other ABC proteins (PMP70, ALDR, P70R) localize to the peroxisomal membrane. The function of this family of peroxisomal membrane proteins is unknown. We used complementation studies to begin analysis of their role in VLCFA β-oxidation and on the peroxisomal membrane. Expression of either ALDP or PMP70 restores VLCFA β-oxidation in X-ALD fibroblasts, indicating overlapping functions. Their expression also restores peroxisome biogenesis in cells that are deficient in the peroxisomal membrane protein Pex2p. Thus it is likely that complex protein interactions are involved in the function and biogenesis of peroxisomal membranes that may contribute to disease heterogeneity.

Original languageEnglish (US)
Pages (from-to)239-247
Number of pages9
JournalHuman molecular genetics
Volume7
Issue number2
DOIs
StatePublished - Feb 1998

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Genetics(clinical)

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