The G(M2) activator protein is an essential cofactor of hexosaminidase
A in the degradation of G(M2) ganglioside and it is responsible for t
he variant AB of G(M2) gangliosidosis in man. In this study G(M2) acti
vator 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 ex
pression of the a subunit of hexosaminidase, thus suggesting a relatio
nship between or subunit and G(M2) activator protein. Furthermore the
results indicate that the expression of G(M2) activator protein is reg
ulated, at least in part, at the transcriptional level. (C) 1994 Acade
mic Press, Inc.