Lysosomal high molecular weight multienzyme complex

被引：0

作者：

Ostrowska, H ^{[1
]}

Krukowska, K ^{[1
]}

Kalinowska, J ^{[1
]}

Orlowska, M ^{[1
]}

Lengiewicz, I ^{[1
]}

机构：

[1] Med Acad Bialystok, Dept Biol, Bialystok, Poland

来源：

CELLULAR & MOLECULAR BIOLOGY LETTERS | 2003年 / 8卷 / 01期

关键词：

protective protein/cathepsin A; acidic glycosidases; multienzyme complex; lysosome;

D O I：

暂无

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Three acidic glycosidases: beta-galactosidase (beta-GAL, EC 3.2.1.23), alpha-neuraminidase (NEUR, sialidase, EC 3.2.1.18), N-acetylaminogalacto-6-sulfate sulfatase (GALNS, EC 3.1.6.4) and serine carboxypepidase cathepsin A (EC 3.4.16.1) form a functional high molecular weight complex in the lysosomes. The major constituent of this complex is cathepsin A, the so-called "lysosomal protective protein" (PPCA). By forming a multienzyme complex, it protects the glycosidases from rapid intralysosomal proteolysis, and it is also required for the intracellular sorting and proteolytic processing of their precursors. In man, a deficiency of cathepsin A leads to a combined deficiency of beta-GAL and NEUR activities, called "galactosialidosis". Multiple mutations identified in the cathepsin A gene are the molecular basis of this lysosomal storage disease. This review describes the structural organization of the lysosomal high molecular weight multienzyme complex and the importance of the protective protein/cathepsin A in physiology and pathology.

引用

页码：19 / 24

页数：6

共 22 条

[1] Lysosomal neuraminidase - Catalytic activation in insect cells is controlled by the protective protein/cathepsin A [J].