An S-cerevisiae peroxisomal transporter, orthologous to the human adrenoleukodystrophy protein, appears to be a heterodimer of two half ABC transporters: Pxa1p and Pxa2p

Abstract

The adrenoleukodystrophy protein (ALDP) and the 70-kDa peroxisomal membrane protein (PMP70) are half ATP binding cassette (ABC) transporters in the peroxisome membrane. ALDP is defective in X-linked adrenoleukodystrophy (ALD), a neurodegenerative disorder with defective peroxisome oxidation of very-long-chain fatty acids.' Mutations in the PMP70 gene were found in two patients with Zellweger syndrome? an inborn error of peroxisome biogenesis, though the role PMP70 plays in this disease is yet unclear. The functions and possible interactions of ALDP and PMP70 in the peroxisomal membrane are not known. To develop a system in which these questions could be addressed, we sought to clone and investigate their yeast orthologue.