The protein coded by the X‐adrenoleukodystrophy gene is a peroxisomal integral membrane protein

FEBS Letters

Aubourg, P.²  Contreras, M.¹  Singh, I.¹  Mandel, J.L.³  Mosser, J.³ 

[1] Department of Pediatrics, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425, USA;INSERM U342, Hospital Saint Vincent de Paul, 75014 Paris, France;CNRS-INSERM U184, 67085 Strasbourg, France

关键词: Peroxisome;    X-adrenoleukodystrophy;    Lignoceric acid oxidation;    X-adrenoleukodystrophy gene product;   

DOI  :  10.1016/0014-5793(94)00400-5

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

The gene for adrenoleukodystrophy (X-ALD), a peroxisomal disease characterized by excessive accumulation of very long-chain (VLC) fatty acids (> C_22;0), has recently been identified by positional cloning, and it is predicted to encode a protein (ALD-P) of 745 amino acids [(1993) Nature 361, 726]. Using Western blot analysis of subcellular organelles purified by isopycnic density gradient centrifugation from X-ALD and control fibroblasts, we show that the monoclonal antibodies directed against ALD-P cross-react with a 75 kDa protein in intact peroxisomes and that ALD-P is an integral component of the peroxisomal membrane. Moreover, no signal for ALD-P was detected in peroxisomes from X-ALD patients with deletion of the ALD gene.

【 授权许可】

Unknown