GM2 activator protein expression in mouse tissues

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / metabolism
  • G(M2) Activator Protein
  • G(M2) Ganglioside / metabolism*
  • Kidney / metabolism
  • Male
  • Mice
  • Mice, Inbred BALB C
  • Proteins / genetics*
  • Proteins / metabolism
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Spleen / metabolism
  • Testis / metabolism
  • Tissue Distribution


  • G(M2) Activator Protein
  • Proteins
  • RNA, Messenger
  • G(M2) Ganglioside