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GM2 activator protein expression in mouse tissues.

Authors
  • Beccari, T1
  • Palmerini, C A
  • Servillo, G
  • Della Fazia, M A
  • Viola Magni, M P
  • Orlacchio, A
  • 1 Dipartimento di Biologia Cellulare e Molecolare, Università degli Studi di Perugia, Italy.
Type
Published Article
Journal
Biochemical and biophysical research communications
Publication Date
Oct 28, 1994
Volume
204
Issue
2
Pages
741–745
Identifiers
PMID: 7980537
Source
Medline
License
Unknown

Abstract

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.

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