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Article

The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis.

Proceedings of the National Academy of Sciences of the United States of America - 20 Jan 1998

Rudenko G, Bonten E, Hol W G, d'Azzo A

Abstract excerpt

Human protective protein/cathepsin A (PPCA), a serine carboxypeptidase, forms a multienzyme complex with beta-galactosidase and neuraminidase and is required for the intralysosomal activity and stability of these two glycosidases. Genetic lesions in PPCA lead to a deficiency of beta-galactosidase and neuraminidase that is manifest as the autosomal recessive lysosomal storage disorder galactosialidosis. Eleven...

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Identifiers

PMID: 9435242
DOI: 10.1073/pnas.95.2.621
PMCID: PMC18470

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The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis.PMID 9435242Read ↗

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Molecular and biochemical analysis of protective protein/cathepsin A mutations: correlation with clinical severity in galactosialidosis.Semantic proximity Read ↗

A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable.Semantic proximity Read ↗

Homologous modeling of the lysosomal protective protein/carboxypeptidase L: structural and functional implications of mutations identified in galactosialidosis patients.Semantic proximity Read ↗

Cathepsin A/protective protein: an unusual lysosomal multifunctional protein.Semantic proximity Read ↗

Lysosomal high molecular weight multienzyme complex.Semantic proximity Read ↗

Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homozygous for the A1184-G transition (Y395C mutation).Semantic proximity Read ↗

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Mouse model for the lysosomal disorder galactosialidosis and correction of the phenotype with overexpressing erythroid precursor cells.Semantic proximity Read ↗

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