Lignoceroyl‐CoASH ligase: enzyme defect in fatty acid β‐oxidation system in X‐linked childhood adrenoleukodystrophy

FEBS Letters

Singh, Inderjit¹  Hashmi, Mazzaz¹  Stanley, Wayne¹ 

[1] Departments of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, USA

关键词: Adrenoleukodystrophy Peroxisome Fatty acid β-Oxidation Lignoceric acid Lignoceroyl-CoA Lignoceroyl-CoA ligase;   

DOI  :  10.1016/0014-5793(86)80256-3

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

We have previously reported that the peroxisomal β-oxidation system for very long chain fatty acids is defective in X-linked childhood adrenoleukodystrophy [(1984) Proc. Natl. Acad. Sci. USA 81, 4203-4207]. In order to elucidate the specific enzyme defect, we examined the oxidation of [1-¹⁴C]lignoceric acid, [1-¹⁴C]lignoceroyl-CoA and (1-¹⁴C)-labelled α,β-unsaturated lignoceroyl-CoA (substrates for the 1st, 2nd, and 3rd steps of the β-oxidation cycle, respectively). These studies suggest that the pathognomonic accumulation of very long chain fatty acids in X-linked childhood ALD may be due to the defective activity of peroxisomal very long chain (lignoceroyl-CoA) acyl-CoA ligase.

【 授权许可】

Unknown