Peroxisomal ABC transporters: Structure, function and role in disease

Masashi Morita, Tsuneo Imanaka*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

149 Scopus citations

Abstract

ATP-binding cassette (ABC) transporters belong to one of the largest families of membrane proteins, and are present in almost all living organisms from eubacteria to mammals. They exist on plasma membranes and intracellular compartments such as the mitochondria, peroxisomes, endoplasmic reticulum, Golgi apparatus and lysosomes, and mediate the active transport of a wide variety of substrates in a variety of different cellular processes. These include the transport of amino acids, polysaccharides, peptides, lipids and xenobiotics, including drugs and toxins. Three ABC transporters belonging to subfamily D have been identified in mammalian peroxisomes. The ABC transporters are half-size and assemble mostly as a homodimer after posttranslational transport to peroxisomal membranes. ABCD1/ALDP and ABCD2/ALDRP are suggested to be involved in the transport of very long chain acyl-CoA with differences in substrate specificity, and ABCD3/PMP70 is involved in the transport of long and branched chain acyl-CoA. ABCD1 is known to be responsible for X-linked adrenoleukodystrophy (X-ALD), an inborn error of peroxisomal β-oxidation of very long chain fatty acids. Here, we summarize recent advances and important points in our advancing understanding of how these ABC transporters target and assemble to peroxisomal membranes and perform their functions in physiological and pathological processes, including the neurodegenerative disease, X-ALD. This article is part of a Special Issue entitled: Metabolic Functions and Biogenesis of Peroxisomes in Health and Disease.

Original languageEnglish
Pages (from-to)1387-1396
Number of pages10
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1822
Issue number9
DOIs
StatePublished - 2012/09

Keywords

  • ABC transporter
  • Acyl-CoA transport
  • Adrenoleukodystrophy
  • Fatty acid β-oxidation
  • Peroxisome targeting

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology

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