CD63-Alix-Rab27a exosome axis is identically influenced in Chediak-Higashi syndrome

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• 作者：Mehdi Hassanpour ; Omid Cheraghi ; Maryam Akbarzadeh…
• 关键词：Chediak ; Higashi ; Exosome biogenesis genes
• 刊名：Comparative Clinical Pathology
• 出版年：2016
• 出版时间：November 2016
• 年：2016
• 卷：25
• 期：6
• 页码：1313-1316
• 全文大小：713 KB
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pathology
  Hematology
  Oncology
  
• 出版者：Springer London
• ISSN：1618-565X
• 卷排序：25

文摘

Chediak-Higashi syndrome is characterized by congenital abnormality in exocytosis activity, and accumulation of intracellular lysosome-like structures different types of cells within the body. Accordingly, the lack of appropriate exocytosis and deficiency in lysosomal trafficking accounts for immune-related hypoactivity. Exosomes are other types of cell-derived vesicles that play key role in cell-cell cross talk. However, the possible effect of LYST gene mutation in exosome trafficking and biogenesis remains to be clarified. Herein, we investigated the expression of CD63-Alix-Rab27a exosome axis in Chediak-Higashi syndrome. Real-time PCR analysis showed a marked reduction in the expression of CD63, Alix, and Rab27a genes in mononuclear and polymorphonuclear cells. In addition to accumulated number of intracellular lysosome, it seems that the biogenesis and trafficking of exosomes were also abolished.