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

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

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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Bibliographic Details
Main Authors: Kotaro Hama, Yuko Fujiwara, Shigeo Takashima, Yasuhiro Hayashi, Atsushi Yamashita, Nobuyuki Shimozawa, Kazuaki Yokoyama
Format: Article
Language:English
Published: Elsevier 2020-04-01
Series:Journal of Lipid Research
Subjects:
fatty acid
fatty acid/metabolism
lipids
lipidomics
peroxisomes
very long-chain fatty acid
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520435023

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