| Title : Expression of cDNA encoding the human protective protein associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases - Galjart_1988_Cell_54_755 |
| Author(s) : Galjart NJ , Gillemans N , Harris A , van der Horst GT , Verheijen FW , Galjaard H , d'Azzo A |
| Ref : Cell , 54 :755 , 1988 |
| Abstract : The "protective protein" is a glycoprotein that associates with lysosomal beta-galactosidase and neuraminidase and is deficient in the autosomal recessive disorder galactosialidosis. We have isolated the cDNA encoding human "protective protein". The clone recognizes a 2 kb mRNA in normal cells that is not evident in fibroblasts of an early infantile galactosialidosis patient. The cDNA directs the synthesis of a 452 amino acid precursor molecule that is processed in vivo to yield mature "protective protein," a heterodimer of 32 kd and 20 kd polypeptides held together by disulfide bridges. This mature form is also biologically functional since it restores beta-galactosidase and neuraminidase activities in galactosialidosis cells. The predicted amino acid sequence of the "protective protein" bears homology to yeast carboxypeptidase Y and the KEX1 gene product. This suggests a protease activity for the "protective protein." |
| ESTHER : Galjart_1988_Cell_54_755 |
| PubMedSearch : Galjart_1988_Cell_54_755 |
| PubMedID: 3136930 |
| Gene_locus related to this paper: human-CTSA |
| Gene_locus related to this paper: human-CTSA |
Galjart NJ, Gillemans N, Harris A, van der Horst GT, Verheijen FW, Galjaard H, d'Azzo A (1988)
Expression of cDNA encoding the human protective protein associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases
Cell
54 :755
Galjart NJ, Gillemans N, Harris A, van der Horst GT, Verheijen FW, Galjaard H, d'Azzo A (1988)
Cell
54 :755