NDLI: Brain microsomal fatty acid elongation is increased in abcd1-deficient mouse during active myelination phase

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Brain microsomal fatty acid elongation is increased in abcd1-deficient mouse during active myelination phase

Content Provider	Springer Nature Link
Author	Morita, Masashi Kawamichi, Misato Shimura, Yusuke Kawaguchi, Kosuke Watanabe, Shiro Imanaka, Tsuneo
Copyright Year	2015
Abstract	The dysfunction of ABCD1, a peroxisomal ABC protein, leads to the perturbation of very long chain fatty acid (VLCFA) metabolism and is the cause of X-linked adrenoleukodystrophy. Abcd1-deficient mice exhibit an accumulation of saturated VLCFAs, such as C26:0, in all tissues, especially the brain. The present study sought to measure microsomal fatty acid elongation activity in the brain of wild-type (WT) and abcd1-deficient mice during the course of development. The fatty acid elongation activity in the microsomal fraction was measured by the incorporation of [2-$^{14}$C]malonyl-CoA into fatty acids in the presence of C16:0-CoA or C20:0-CoA. Cytosolic fatty acid synthesis activity was completely inhibited by the addition of N-ethylmaleimide (NEM). The microsomal fatty acid elongation activity in the brain was significantly high at 3 weeks after birth and decreased substantially at 3 months after birth. Furthermore, we detected two different types of microsomal fatty acid elongation activity by using C16:0-CoA or C20:0-CoA as the substrate and found the activity toward C20:0-CoA in abcd1-deficient mice was higher than the WT 3-week-old animals. These results suggest that during the active myelination phase the microsomal fatty acid elongation activity is stimulated in abcd1-deficient mice, which in turn perturbs the lipid composition in myelin.
Starting Page	1359
Ending Page	1367
Page Count	9
File Format	PDF
ISSN	08857490
Journal	Metabolic Brain Disease
Volume Number	30
Issue Number	6
e-ISSN	15737365
Language	English
Publisher	Springer US
Publisher Date	2015-06-25
Publisher Place	New York
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	ABCD1 X-linked adrenoleukodystrophy Very long chain fatty acid Microsomal fatty acid elongation Neurosciences Neurology Metabolic Diseases Biochemistry Oncology
Content Type	Text
Resource Type	Article
Subject	Biochemistry Neurology (clinical) Cellular and Molecular Neuroscience

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