IUPHAR/BPS Guide to Pharmacology CITE
ABCD subfamily of peroxisomal ABC transporters (version 2019.4) in the IUPHAR/BPS Guide to Pharmacology Database
- University of Amsterdam, The Netherlands
Peroxisomes are indispensable organelles in higher eukaryotes. They are essential for the oxidation of a wide variety of metabolites, which include: saturated, monounsaturated and polyunsaturated fatty acids, branched-chain fatty acids, bile acids and dicarboxylic acids . However, the peroxisomal membrane forms an impermeable barrier to these metabolites. The mammalian peroxisomal membrane harbours three ATP-binding cassette (ABC) half-transporters, which act as homo- and/or heterodimers to transport these metabolites across the peroxisomal membrane.
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ABCD subfamily of peroxisomal ABC transporters
- Coelho D, Kim JC, Miousse IR, Fung S, du Moulin M, Buers I, Suormala T, Burda P, Frapolli M and Stucki M et al.. (2012) Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism. Nat. Genet. 44: 1152-5 [PMID:22922874]
- Ferdinandusse S, Jimenez-Sanchez G, Koster J, Denis S, Van Roermund CW, Silva-Zolezzi I, Moser AB, Visser WF, Gulluoglu M and Durmaz O et al.. (2015) A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3. Hum. Mol. Genet. 24: 361-70 [PMID:25168382]
- Kemp S, Theodoulou FL and Wanders RJ. (2011) Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance. Br. J. Pharmacol. 164: 1753-66 [PMID:21488864]
- van Roermund CW, Visser WF, Ijlst L, van Cruchten A, Boek M, Kulik W, Waterham HR and Wanders RJ. (2008) The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters. FASEB J. 22: 4201-8 [PMID:18757502]
- van Roermund CW, Visser WF, Ijlst L, Waterham HR and Wanders RJ. (2011) Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation. Biochim. Biophys. Acta 1811: 148-52 [PMID:21145416]