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Theses/Dissertations

University of Massachusetts Amherst

2014

Biochemistry, Biophysics, and Structural Biology

Cathepsin A

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Full-Text Articles in Life Sciences

Structural Biology And Pharmacology Of Human Cathepsin A And Neuraminidase 1, Nilima Kolli Aug 2014

Structural Biology And Pharmacology Of Human Cathepsin A And Neuraminidase 1, Nilima Kolli

Doctoral Dissertations

Human cathepsin A (also known as Protective Protein/Cathepsin A, PPCA; E.C. 3.4.16.5) is a lysosomal serine carboxypeptidase. Cathepsin A is also involved in a complex with two other lysosomal enzymes: lysosomal neuraminidase (NEU1, E.C. 3.2.1.18) and β-galactosidase (GLB1, E.C. 3.2.1.23). Deficiency in cathepsin A and NEU1 result in the lysosomal storage diseases, galactosialidosis and sialidosis respectively. Deficiency in GLB1 results in GM1 gangliosidosis and Morquio B diseases.

Cathepsin A protease activity is spatially regulated by activation of the inactive precursor form to the mature form in ...