一個十天半大之HspB7LacZ/+基因改造小鼠胚胎經由X-gal 染色處理後,呈現出小熱休克蛋白B7組織特異性之表現

The Cardiac Specific Expression of Small Heat Shock Protein B7 (HspB7) is Present in E10.5 HspB7LacZ/+ Mouse Embryo with X-gal Staining
一個十天半大之HspB7LacZ/+基因改造小鼠胚胎經由X-gal 染色處理後,呈現出小熱休克蛋白B7組織特異性之表現

小熱休克蛋白B7是B群小熱休克蛋白家族的成員之一,它在心肌細胞和骨骼肌細胞中表達。 藉由基因改造小鼠研究發現,心臟或骨骼肌失去小熱休克蛋白B7會分別導致骨骼肌 (1) 和心臟間盤 (2) 的肌纖維結構破壞失能而致死。此外,它還會導致心肌和骨骼肌肉中之絲蛋白(Filamin C, FLNC)變性聚集沉澱或失去活性。絲蛋白和HspB7存在有二者之相互作用,顯現出小熱休克蛋白B7在維持絲蛋白的結構和功能中發揮著其伴侶作用。

HSPB7, a member of the small heat-shock protein (HSPB) family, is expressed in the cardiomyocytes and skeletal muscle. Loss of HSPB7 results in the destruction of myofiber structure in the skeletal muscle (1) and the interclatedisk of heart (2), respectively. In addition, it also causes overexpression and aggregation of Filamin C (FLNC) in both mutant tissues. In addition, the interaction of active FLNC and HspB7 is observed in living cells, suggesting HspB7 plays a chaperone role in maintaining FLNC structure and function.

論文全文 Article Link:

  1. https://doi.org/10.1242/jcs.179887
  2. https://doi.org/10.1371/journal.pgen.1006984