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ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1–3 and ABCD4 in Subcellular Localization, Function, and Human Disease
Joint Authors
Kawaguchi, Kosuke
Morita, Masashi
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-09-28
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
ATP-binding cassette (ABC) transporters are one of the largest families of membrane-bound proteins and transport a wide variety of substrates across both extra- and intracellular membranes.
They play a critical role in maintaining cellular homeostasis.
To date, four ABC transporters belonging to subfamily D have been identified.
ABCD1–3 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 B12 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 B12 deficiency, respectively.
In this review, the targeting mechanism and physiological functions of the ABCD transporters are summarized along with the related disease.
American Psychological Association (APA)
Kawaguchi, Kosuke& Morita, Masashi. 2016. ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1–3 and ABCD4 in Subcellular Localization, Function, and Human Disease. BioMed Research International،Vol. 2016, no. 2016, pp.1-11.
https://search.emarefa.net/detail/BIM-1098549
Modern Language Association (MLA)
Kawaguchi, Kosuke& Morita, Masashi. ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1–3 and ABCD4 in Subcellular Localization, Function, and Human Disease. BioMed Research International No. 2016 (2016), pp.1-11.
https://search.emarefa.net/detail/BIM-1098549
American Medical Association (AMA)
Kawaguchi, Kosuke& Morita, Masashi. ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1–3 and ABCD4 in Subcellular Localization, Function, and Human Disease. BioMed Research International. 2016. Vol. 2016, no. 2016, pp.1-11.
https://search.emarefa.net/detail/BIM-1098549
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1098549