产品描述 | Lignoceric acid-d47 is intended for use as an internal standard for the quantification of lignoceric acid by GC- or LC-MS. Lignoceric acid is a 24-carbon saturated (24:0) fatty acid. In mammals, it is synthesized during brain development and is found in cerebrosides.1 The deficient peroxisomal oxidation of very-long-chain fatty acids, including lignoceric acid, contributes to certain syndromes, including Zellweger cerebro-hepato-renal syndrome and X chromosome-linked adrenoleukodystrophy.2 Lignoceric acid is also a by-product of lignin production. References 1. Bourre, J.M., Paturneau-Jouas, M.Y., Daudu, O.L., et al. Lignoceric acid biosynthesis in the developing brain. Activities of mitochondrial acetyl-CoA-dependent synthesis and microsomal malonyl-CoA chain-elongating system in relation to myelination. Eur. J. Biochem. 72(1), 41-47 (1977). 2. Singh, I., Moser, A.E., Goldfischer, S., et al. Lignoceric acid is oxidized in the peroxisome: Implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy. Proc. Natl. Acad. Sci. USA 81(13), 4203-4207 (1984). |