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Protective protein for beta-glactosidase (glactosialidosis) also known as Cathepsin A is a serine acid carboxypeptidase present lysosomes where it forms a fully functional and stable high molecular weight complex with beta-D-glactosidase and N-acetyl-alpha-neuraminindase. Cathepsin A functions in intracellular routing, lysosomal localization, activation of neuraminidase and protection of beta-glactosidase against rapid proteolytic degradation. Its deficiency in man leads to the lysosomal storage disorder galactosialidosis, characterized by the deficiency of both neurominidase and beta-galactosidase. The 54 kD precursor is processed into 32 kD and 20 kD subunits that form a heterodimer.
|Gene Name:||Cathepsin A|
|Family/Subfamily:||Protease , Serine S10|
|Synonyms:||CTSA, Beta-galactosidase 2, Carboxypeptidase-L, Carboxypeptidase C, GSL, GLB2, Lysosomal carboxypeptidase A, PPCA, Protective protein cathepsin A, Protective protein, Urinary kininase, PPGB, Carboxypeptidase L, Cathepsin A, Cathepsin A (PPCA), Deamidase, Lysosomal protective protein, NGBE|
|Target Sequences:||NM_000308 NP_000299.2 P10619|
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