Normal lysosomal morphology and function in @entity32329 -deficient XXXX .

@entity19 @entity9674 @entity43005 @entity32329
Lysosomal membranes contain two highly glycosylated proteins, designated @entity32329 and @entity9674 , as major components. @entity32329 and @entity9674 are structurally related. To investigate the physiological role of @entity32329 , we have generated @entity19 deficient for this protein. @entity32329 -deficient @entity19 are viable and fertile. In @entity32329 -deficient brain, a mild regional astrogliosis and altered immunoreactivity against @entity43005 was observed. Histological and ultrastructural analyses of all other tissues did not reveal abnormalities. Lysosomal properties, such as enzyme activities, lysosomal pH, osmotic stability, density, shape, and subcellular distribution were not changed in comparison with controls. Western blot analyses of @entity32329 -deficient and heterozygote tissues revealed an up-regulation of the @entity9674 protein pointing to a compensatory effect of @entity9674 in response to the @entity32329 deficiency. The increase of @entity9674 was neither correlated with an increase in the level of @entity9674 mRNAs nor with increased half-life time of @entity9674 . This findings suggest a translational regulation of @entity9674 expression.
