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对一种高分子量蛋白质进行免疫学鉴定,该蛋白质为杜氏肌营养不良基因产物的候选蛋白。

Immunological identification of a high molecular weight protein as a candidate for the product of the Duchenne muscular dystrophy gene.

作者信息

Kao L, Krstenansky J, Mendell J, Rammohan K W, Gruenstein E

机构信息

Department of Biochemistry and Molecular Biology, University of Cincinnati College of Medicine, OH 45267-0522.

出版信息

Proc Natl Acad Sci U S A. 1988 Jun;85(12):4491-5. doi: 10.1073/pnas.85.12.4491.

Abstract

An oligopeptide was synthesized based on translation of the nucleotide sequence of the putative exon region of clone pERT87-25 from the gene for Duchenne muscular dystrophy. Immunization of rabbits with this oligopeptide induced the formation of antibodies directed against a protein present in human, rat, and rabbit skeletal muscle. This protein, which is missing in the skeletal muscle of two patients with Duchenne muscular dystrophy, has a molecular mass of approximately equal to 320-420 kDa and is clearly different from the putative Duchenne muscular dystrophy-related protein nebulin. The data suggest that this 320- to 420-kDa protein is produced by the Duchenne muscular dystrophy gene.

摘要

基于杜兴氏肌营养不良症基因克隆pERT87 - 25推定外显子区域的核苷酸序列翻译,合成了一种寡肽。用这种寡肽免疫兔子,诱导产生了针对人、大鼠和兔子骨骼肌中一种蛋白质的抗体。在两名杜兴氏肌营养不良症患者的骨骼肌中缺失的这种蛋白质,其分子量约为320 - 420 kDa,且明显不同于推定的与杜兴氏肌营养不良症相关的蛋白质伴肌动蛋白。数据表明,这种320至420 kDa的蛋白质是由杜兴氏肌营养不良症基因产生的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7391/280456/5d2303b8aa28/pnas00264-0386-a.jpg

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