Targeted disruption of the mouse (cid:97) A-crystallin gene induces cataract and cytoplasmic inclusion bodies containing the small heat shock protein (cid:97) B-crystallin
Targeted disruption of the mouse (cid:97) A-crystallin gene induces cataract and cytoplasmic inclusion bodies containing the small heat shock protein (cid:97) B-crystallin
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靶向破坏小鼠 (cid:97) A-晶状体蛋白基因可诱导白内障和含有小热休克蛋白 (cid:97) B-晶状体蛋白的细胞质包涵体
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发表时间：
1997
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通讯作者：
Wawrousek
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作者：
James;P.;Brady;D. Garland;Y. Duglas;Tabor;W.;G. Robison;A. Groome;Eric;F.;Wawrousek
(cid:97) A-晶状体蛋白 ( (cid:97) A) 和 (cid:97) B-晶状体蛋白 ( (cid:97) B) 是脊椎动物眼睛晶状体的主要蛋白质。在体外，(cid:97)-晶状体蛋白作为高分子质量聚集体被分离在一起，表现出许多特性，其中最有趣的是它们作为其他蛋白质的分子伴侣的能力。在这里，我们开始通过产生具有 (cid:97) A 基因靶向破坏的小鼠来检查 (cid:97)-晶状体蛋白的体内功能。根据蛋白质凝胶电泳和免疫印迹分析，被破坏的等位基因纯合的小鼠的晶状体中没有产生可检测到的 (cid:97) A。最初，（cid：97）A缺陷晶状体看起来结构正常，但它们比野生型同窝动物的晶状体小。 (cid:97) A (cid:50) / (cid:50) 镜片会出现从细胞核开始的混浊，并随着年龄的增长逐渐发展为全身混浊。光学和透射电子显微镜揭示了中央晶状体纤维细胞中存在致密的包涵体。内含物与 (cid:97) B 抗体反应强烈，但与 (cid:98) 抗体反应不显着
(cid:97) A-crystallin ( (cid:97) A) and (cid:97) B-crystallin ( (cid:97) B) are among the predominant proteins of the vertebrate eye lens. In vitro , the (cid:97) -crystallins, which are isolated together as a high molecular mass aggregate, exhibit a number of properties, the most interesting of which is their ability to function as molecular chaperones for other proteins. Here we begin to examine the in vivo functions of (cid:97) -crystallin by generating mice with a targeted disruption of the (cid:97) A gene. Mice that are homozygous for the disrupted allele produce no detectable (cid:97) A in their lenses, based on protein gel electrophoresis and immunoblot analysis. Initially, the (cid:97) A-deficient lenses appear structurally normal, but they are smaller than the lenses of wild-type littermates. (cid:97) A (cid:50) / (cid:50) lenses develop an opacification that starts in the nucleus and progresses to a general opacification with age. Light and transmission electron microscopy reveal the presence of dense inclusion bodies in the central lens fiber cells. The inclusions react strongly with antibodies to (cid:97) B but not significantly with antibodies to (cid:98)