Mouse "protective protein". cDNA cloning, sequence comparison, and expression.
作者： N J Galjart , N Gillemans , D Meijer , A d'Azzo
DOI: 10.1016/S0021-9258(19)39616-4
关键词:
摘要: The "protective protein" is the glycoprotein that forms a complex with lysosomal enzymes beta-galactosidase and neuraminidase. Its deficiency in man leads to metabolic storage disorder galactosialidosis. primary structure of human protective protein, deduced from its cloned cDNA, shows homology yeast serine carboxypeptidases. We have isolated full-length cDNA encoding murine protein. nucleotide sequences as well predicted amino acid are highly conserved between mouse. Domains important for protease function completely identical two proteins. Both mouse mature proteins covalently bind radiolabeled diisopropyl fluorophosphate. Transient expression COS-1 cells yields protein precursor 54 kDa, size characteristic glycosylated form. This cDNA-encoded precursor, endocytosed by galactosialidosis fibroblasts, processed into 32- 20-kDa heterodimer corrects neuraminidase activities. A tissue-specific mRNA observed when total RNA different organs analyzed on Northern blots.
cabdirect.org
researchgate.net 
sci-hub.st 参考文章(34)
Rikimaru Hayashi, Stanford Moore, William H. Stein, Serine at the active center of yeast carboxypeptidase. Journal of Biological Chemistry. ,vol. 248, pp. 8366- 8369 ,(1973) , 10.1016/S0021-9258(19)43142-6
L.E. Quintern, E.H. Schuchman, O. Levran, M. Suchi, K. Ferlinz, H. Reinke, K. Sandhoff, R.J. Desnick, Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts. The EMBO Journal. ,vol. 8, pp. 2469- 2473 ,(1989) , 10.1002/J.1460-2075.1989.TB08382.X
D. Meijer, A. Hermans, M. von Lindern, T. van Agthoven, A. de Klein, P. Mackenbach, A. Grootegoed, D. Talarico, G. Della Valle, G. Grosveld, Molecular characterization of the testis specific c-abl mRNA in mouse The EMBO Journal. ,vol. 6, pp. 4041- 4048 ,(1987) , 10.1002/J.1460-2075.1987.TB02749.X
A. Hasilik, E.F. Neufeld, BIOSYNTHESIS OF LYSOSOMAL ENZYMES IN FIBROBLASTS. SYNTHESIS AS PRECURSORS OF HIGHER MOLECULAR WEIGHT Journal of Biological Chemistry. ,vol. 255, pp. 4937- 4945 ,(1980) , 10.1016/S0021-9258(19)85585-0
G T J van der Horst, N J Galjart, A d'Azzo, H Galjaard, F W Verheijen, Identification and in Vitro Reconstitution of Lysosomal Neuraminidase from Human Placenta Journal of Biological Chemistry. ,vol. 264, pp. 1317- 1322 ,(1989) , 10.1016/S0021-9258(19)85088-3
H Morreau, N.J. Galjart, N Gillemans, R Willemsen, G.T. van der Horst, A d'Azzo, Alternative splicing of beta-galactosidase mRNA generates the classic lysosomal enzyme and a beta-galactosidase-related protein. Journal of Biological Chemistry. ,vol. 264, pp. 20655- 20663 ,(1989) , 10.1016/S0021-9258(19)47114-7
Rikimaru Hayashi, Stanford Moore, William H. Stein, Carboxypeptidase from yeast. Large scale preparation and the application to COOH-terminal analysis of peptides and proteins. Journal of Biological Chemistry. ,vol. 248, pp. 2296- 2302 ,(1973) , 10.1016/S0021-9258(19)44109-4
R L Proia, A d'Azzo, E F Neufeld, Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblasts. Journal of Biological Chemistry. ,vol. 259, pp. 3350- 3354 ,(1984) , 10.1016/S0021-9258(17)43301-1
Frans W. VERHEIJEN, Silvia PALMERI, Andre T. HOOGEVEEN, Hans GALJAARD, Human placental neuraminidase FEBS Journal. ,vol. 149, pp. 315- 322 ,(1985) , 10.1111/J.1432-1033.1985.TB08928.X
A T Hoogeveen, F W Verheijen, H Galjaard, The relation between human lysosomal beta-galactosidase and its protective protein. Journal of Biological Chemistry. ,vol. 258, pp. 12143- 12146 ,(1983) , 10.1016/S0021-9258(17)44147-0