Adrenoleukodystrophy gene encodes an 80 kDa membrane protein.
Adrenoleukodystrophy gene encodes an 80 kDa membrane protein.
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肾上腺脑白质营养不良基因编码 80 kDa 膜蛋白。
DOI：
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发表时间：
1994
期刊：
影响因子：
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通讯作者：
T. Iwaki
中科院分区：
文献类型：
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作者：
T. Kobayashi;T. Yamada;T. Yasutake;N. Shinnoh;I. Goto;T. Iwaki
从X-连锁肾上腺脑白质营养不良(ALD)致病基因的cDNA中合成C端肽的抗体用于鉴定ALD基因的产物。该抗体在对照成纤维细胞中与80 kDa条带蛋白反应，而在1例ALD患者(#163)的成纤维细胞中未检测到条带，Northern印迹分析未检测到ALD基因的mRNA。经Western印迹检测，293T细胞表达出一种新的80 kDa蛋白。在免疫细胞学研究中，正常成纤维细胞的染色呈点状。而在#163细胞中未见点状染色。这些数据表明，ALD基因编码一个80 kDa的膜蛋白。
An antibody against the synthetic C-terminal peptides deduced from the cDNA of the gene responsible for X-linked adrenoleukodystrophy (ALD) was produced to characterize the product of the ALD gene. The antibody reacted with the 80 kDa band protein in control fibroblasts, while no bands were detected in the fibroblasts from a patient with ALD (#163), in which mRNA of the ALD gene was undetectable based on Northern blot analysis. The 293T cells transfected with the full-coding cDNA inserted in the expression vector produced a new 80 kDa protein, as detected by Western blot. In an immunocytological study, the staining was in a punctate pattern, in the normal fibroblasts. However, there was no punctate staining in the #163 cells. These data thus indicate that the ALD gene encodes an 80 kDa membrane protein.