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They play a critical role in maintaining cellular homeostasis. To date, four ABC transporters belonging to subfamily D have been identified. ABCD1\u20133 and ABCD4 are localized to peroxisomes and lysosomes, respectively. ABCD1 and ABCD2 are involved in the transport of long and very long chain fatty acids (VLCFA) or their CoA-derivatives into peroxisomes with different substrate specificities, while ABCD3 is involved in the transport of branched chain acyl-CoA into peroxisomes. On the other hand, ABCD4 is deduced to take part in the transport of vitamin B<jats:sub>12<\/jats:sub>from lysosomes into the cytosol. It is well known that the dysfunction of ABCD1 results in X-linked adrenoleukodystrophy, a severe neurodegenerative disease. Recently, it is reported that ABCD3 and ABCD4 are responsible for hepatosplenomegaly and vitamin B<jats:sub>12<\/jats:sub>deficiency, respectively. In this review, the targeting mechanism and physiological functions of the ABCD transporters are summarized along with the related disease.<\/jats:p>","DOI":"10.1155\/2016\/6786245","type":"journal-article","created":{"date-parts":[[2016,9,28]],"date-time":"2016-09-28T21:11:53Z","timestamp":1475097113000},"page":"1-11","source":"Crossref","is-referenced-by-count":72,"title":["ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1\u20133 and ABCD4 in Subcellular Localization, Function, and Human Disease"],"prefix":"10.1155","volume":"2016","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5115-7169","authenticated-orcid":true,"given":"Kosuke","family":"Kawaguchi","sequence":"first","affiliation":[{"name":"Department of Biological Chemistry, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, 2630 Sugitani, Toyama 930-0194, 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