Human Molecular Genetics, Vol 7, 239-247, Copyright © 1998 by Oxford University Press
LT Braiterman, S Zheng, PA Watkins, MT Geraghty, G Johnson, MC McGuinness, AB Moser and KD Smith
X-Linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder
characterized by reduced peroxisomal very long chain fatty acid (VLCFA)
beta-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 beta-oxidation and on the peroxisomal membrane. Expression of either
ALDP or PMP70 restores VLCFA beta- 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.
ARTICLES
Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins
Kennedy Krieger Research Institute, 707 North Broadway, Baltimore, MD 21205, USA. lita@welchlink.welch.jhu.edu
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