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J. Biol. Chem., Vol. 258, Issue 20, 12143-12146, 10, 1983
AT Hoogeveen, FW Verheijen and H Galjaard
Cultured skin fibroblasts from patients with the lysosomal storage disease
galactosialidosis lack a 54-kDa protein which is a precursor of 32-kDa and
20-kDa proteins, which immunoprecipitate with human anti-
beta-galactosidase antiserum. The lack of a 32-kDa "protective protein"
results in a combined deficiency of beta-galactosidase and sialidase. The
mechanism of protection of lysosomal beta-galactosidase against proteolytic
degradation is elucidated by sucrose density gradient centrifugation and
immunoprecipitation studies. In normal fibroblasts at the low
intralysosomal pH, more than 85% of beta-galactosidase exists as a high
molecular weight (600-700 kDa) multimer and about 10% as a monomer of
64-kDa. In mutant cells from galactosialidosis patients, the residual
enzyme activity, about 10%, is present as a monomer and no multimer exists.
After addition of the 54-kDa precursor form of the protective protein, the
density pattern of beta- galactosidase in galactosialidosis cells is
normalized. Immunoprecipitation studies after sucrose density gradient
centrifugation on homogenate and on purified beta-galactosidase from normal
fibroblasts show that the protective protein is associated only with the
multimeric form of beta-galactosidase. We propose that intralysosomal
protection against proteolysis of beta-galactosidase and sialidase is
accomplished by aggregation into a high molecular weight complex consisting
of multimeric beta-galactosidase, sialidase, and protective protein. The
genetic deficiency of the latter, as in galactosialidosis, results in a
rapid degradation of monomeric beta- galactosidase and a loss of sialidase
activity.
The relation between human lysosomal beta-galactosidase and its protective protein
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