GM2激活蛋白在小鼠组织中的表达。
GM2 activator protein expression in mouse tissues.
作者信息
Beccari T, Palmerini C A, Servillo G, Della Fazia M A, Viola Magni M P, Orlacchio A
机构信息
Dipartimento di Biologia Cellulare e Molecolare, Università degli Studi di Perugia, Italy.
出版信息
Biochem Biophys Res Commun. 1994 Oct 28;204(2):741-5. doi: 10.1006/bbrc.1994.2521.
The GM2 activator protein is an essential cofactor of hexosaminidase A in the degradation of GM2 ganglioside and it is responsible for the variant AB of GM2 gangliosidosis in man. In this study GM2 activator protein and its mRNA were determined in different mouse tissues. It was found that this protein is expressed mostly in the spleen and testis followed by brain and kidney which represent the main source in man. It is also interesting that in mouse testis there is a higher expression of the alpha subunit of hexosaminidase, thus suggesting a relationship between alpha subunit and GM2 activator protein. Furthermore the results indicate that the expression of GM2 activator protein is regulated, at least in part, at the transcriptional level.
GM2激活蛋白是己糖胺酶A降解GM2神经节苷脂过程中必需的辅助因子，它与人类GM2神经节苷脂贮积症的AB变异型有关。在本研究中，对不同小鼠组织中的GM2激活蛋白及其mRNA进行了测定。结果发现，该蛋白主要在脾脏和睾丸中表达，其次是大脑和肾脏，而在人类中这些组织是主要来源。有趣的是，在小鼠睾丸中己糖胺酶α亚基的表达较高，这表明α亚基与GM2激活蛋白之间存在某种关系。此外，结果表明GM2激活蛋白的表达至少部分受转录水平的调控。
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