Merck
  • Home
  • Search Results
  • Brain microsomal fatty acid elongation is increased in abcd1-deficient mouse during active myelination phase.

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

Metabolic brain disease (2015-06-26)
Masashi Morita, Misato Kawamichi, Yusuke Shimura, Kosuke Kawaguchi, Shiro Watanabe, Tsuneo Imanaka
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.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Hexane, puriss. p.a., ACS reagent, reag. Ph. Eur., ≥99% (GC)
Sigma-Aldrich
Hexane, ReagentPlus®, ≥99%
Sigma-Aldrich
Hexane, Laboratory Reagent, ≥95%
Supelco
Sucrose, analytical standard
Sigma-Aldrich
Sucrose, JIS special grade
Sigma-Aldrich
Hexane, SAJ first grade, ≥95.0%
Sigma-Aldrich
Hexane, ≥96.0%, suitable for residual phthalate analysis
Sigma-Aldrich
Hexane, JIS 300, ≥96.0%, for residue analysis
Sigma-Aldrich
Hexane, JIS 1000, ≥96.0%, for residue analysis
Supelco
Hexane, suitable for HPLC, ≥95%
Sigma-Aldrich
Hexane, suitable for HPLC
Sigma-Aldrich
Hexane, HPLC Plus, for HPLC, GC, and residue analysis, ≥95%
Sigma-Aldrich
Sucrose, SAJ first grade
Sigma-Aldrich
Hexane, JIS special grade, ≥96.0%
Sigma-Aldrich
Hexane, anhydrous, 95%
Sigma-Aldrich
Sucrose, BioUltra, for molecular biology, ≥99.5% (HPLC)
Sigma-Aldrich
Sucrose, puriss., meets analytical specification of Ph. Eur., BP, NF
Sigma-Aldrich
Hexane, puriss. p.a., ACS reagent, ≥99.0% (GC)
Sigma-Aldrich
Hexane, for residue analysis, JIS 5000
Sigma-Aldrich
Sucrose, ≥99.5% (GC)
Sigma-Aldrich
Sucrose, for molecular biology, ≥99.5% (GC)
Sigma-Aldrich
Sucrose, ACS reagent
Sigma-Aldrich
Sucrose, BioXtra, ≥99.5% (GC)
Sigma-Aldrich
Sucrose, BioReagent, suitable for cell culture, suitable for insect cell culture, ≥99.5% (GC)
Sigma-Aldrich
Methanol-12C, 99.95 atom % 12C
Supelco
Methanol solution, contains 0.10 % (v/v) formic acid, UHPLC, suitable for mass spectrometry (MS), ≥99.5%
Sigma-Aldrich
Sucrose, AR
Sigma-Aldrich
Hexane, AR, ≥99%
Sigma-Aldrich
Hydrogen chloride solution, 1.0 M in diethyl ether
Sigma-Aldrich
Hydrochloric acid, ACS reagent, 37%