| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 1904913 | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2012 | 10 Pages |
Abstract
⺠Peroxisomal ABC transporters (ABCD1-3) are half-size and assemble mostly as a homodimer. ⺠Peroxisomal ABC transporters are posttranslationally transported to peroxisomal membranes. ⺠Peroxisomal ABC transporters are involved in the transport of a variety of acyl-CoAs. ⺠Dysfunction of ABCD1 causes X-linked adrenoleukodystrophy, a neurodegenerative disorder. ⺠ABCD1 and 2 are responsible for axonal integrity and myelination in central nervous system.
Keywords
trihydroxycholestanoic acidX-ALDadrenoleukodystrophyCCALDVLCFAGFPX-linked adrenoleukodystrophyNBDAMNTHCATMDDHCAABC transporteriPSROSFatty acid β-oxidationABCDadrenomyeloneuropathydocosahexaenoic aciddihydroxycholestanoic acidPolyunsaturated fatty acidPUFAvery long chain fatty acidsnucleotide-binding domaintransmembrane domainCNSDHAinduced pluripotent stemcentral nervous systemgreen fluorescent proteinSingle nucleotide polymorphismSNPReactive oxygen species
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Authors
Masashi Morita, Tsuneo Imanaka,