首页    期刊浏览 2024年07月06日 星期六
登录注册

文章基本信息

  • 标题:Arylsulfatase A deficiency causes seminolipid accumulation and a lysosomal storage disorder in Sertoli cells
  • 本地全文:下载
  • 作者:Hongbin Xu ; Kessiri Kongmanas ; Suraj Kadunganattil
  • 期刊名称:JLR Papers In Press
  • 印刷版ISSN:0022-2275
  • 电子版ISSN:1539-7262
  • 出版年度:2011
  • 卷号:52
  • 期号:12
  • 页码:2187-2197
  • DOI:10.1194/jlr.M019661
  • 语种:English
  • 出版社:American Society for Biochemistry and Molecular Biology
  • 摘要:Sulfogalactosylglycerolipid (SGG) is the major sulfoglycolipid of male germ cells. During spermatogenesis, apoptosis occurs in >50% of total germ cells. Sertoli cells phagocytose these apoptotic germ cells and degrade their components using lysosomal enzymes. Here we demonstrated that SGG was a physiological substrate of Sertoli lysosomal arylsulfatase A (ARSA). SGG accumulated in Sertoli cells of Arsa −/− mice, and at 8 months of age, this buildup led to lysosomal swelling and other cellular abnormalities typical of a lysosomal storage disorder. This disorder likely compromised Sertoli cell functions, manifesting as impaired spermatogenesis and production of sperm with near-zero fertilizing ability in vitro. Fecundity of Arsa −/− males was thus reduced when they were older than 5 months. Sperm SGG is known for its roles in fertilization. Therefore, the minimal sperm fertilizing ability of 8-month-old Arsa −/− males may be explained by the 50% reduction of their sperm SGG levels, a result that was also observed in testicular germ cells. These unexpected decreases in SGG levels might be partly due to depletion of the backbone lipid palmitylpalmitoylglycerol that is generated from the SGG degradation pathway in Sertoli cells and normally recycled to new generations of primary spermatocytes for SGG synthesis.
  • 关键词:spermatogenesis ; lysosomes ; electron microscopy
国家哲学社会科学文献中心版权所有