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Hexacosenoyl-CoA is the most abundant very long-chain acyl-CoA in ATP binding cassette transporter D1-deficient cells

X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder caused by deleterious mutations in the ABCD1 gene. The ABCD1 protein transports very long-chain FAs (VLCFAs) from the cytosol into the peroxisome where the VLCFAs are degraded through β-oxidation. ABCD1 dysfunction leads to VLCFA accumul...

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Pubblicato in:J Lipid Res
Autori principali: Hama, Kotaro, Fujiwara, Yuko, Takashima, Shigeo, Hayashi, Yasuhiro, Yamashita, Atsushi, Shimozawa, Nobuyuki, Yokoyama, Kazuaki
Natura: Artigo
Lingua:Inglês
Pubblicazione: The American Society for Biochemistry and Molecular Biology 2020
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC7112142/
https://ncbi.nlm.nih.gov/pubmed/32075856
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.P119000325
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