Lysosomal high molecular weight multienzyme complex

• Autorzy: Ostrowska H , Krukowska K. , Kalinowska J. , Orlowska M. , Lengiewicz I.
• Języki publikacji: EN

Abstrakty

EN

Three acidic glycosidases: β-galactosidase (β-GAL, EC 3.2.1.23), α-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 β-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.

Słowa kluczowe

EN

alpha-neuraminidase; sialidase; procective protein; lysosome; acidic glycosidase; beta-galactosidase; multienzyme complex; cathepsin A; N-acetylaminogalacto-6-sulphate sulphatase

• Wydawca: -
• Czasopismo: Cellular and Molecular Biology Letters
• Rocznik: 2003
• Tom: 08
• Numer: 1
• Opis fizyczny: p.19-24

Twórcy

autor

Ostrowska H

• Medical Academy of Bialystok, Bialystok, Poland

autor

Krukowska K.

autor

Kalinowska J.

autor

Orlowska M.

autor

Lengiewicz I.

Bibliografia

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