The peroxisomal membrane forms a permeability barrier for a wide variety of
metabolites required for and formed during fatty acid beta-oxidation. To c
ommunicate with the cytoplasm and mitochondria, peroxisomes need dedicated
proteins to transport such hydrophilic molecules across their membranes. Ge
netic and biochemical studies in the yeast Sacchauomyces cerevisiae have id
entified enzymes for redox shuttles as well as the first peroxisomal membra
ne transporter. This peroxisomal ATP-binding cassette transporter (Pat) is
highly homologous to the gene mutated in X-linked adrenoleukodystrophy (X-A
LD). The yeast Pat is required for import of activated fatty acids into per
oxisomes suggesting that this is the primary defect in X-ALD. (C) 2000 Else
vier Science B.V. All rights reserved.