FunctionProtective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.
Involvement in diseaseDefects in CTSA are the cause of galactosialidosis (GSL) [MIM:256540]. A lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, secondary to a defect in cathepsin A. All patients have clinical manifestations typical of a lysosomal disorder, such as coarse facies, cherry red spots, vertebral changes, foam cells in the bone marrow, and vacuolated lymphocytes. Three phenotypic subtypes are recognized. The early infantile form is associated with fetal hydrops, edema, ascites, visceromegaly, skeletal dysplasia, and early death. The late infantile type is characterized by hepatosplenomegaly, growth retardation, cardiac involvement, and a normal or mildly affected mental state. The juvenile/adult form is characterized by myoclonus, ataxia, angiokeratoma, mental retardation, neurologic deterioration, absence of visceromegaly, and long survival.
Sequence similaritiesBelongs to the peptidase S10 family.
Neuraminidase deficiency with beta-galactosidase deficiency antibody
PPCA deficiency antibody
Protective protein cathepsin A antibody
Protective protein for beta galactosidase antibody
Protective protein for beta-galactosidase antibody
Protective protein/cathepsin A deficiency antibody
urinary kininase antibody
Anti-Protective protein/Cathepsin A (PPCA) antibody images
Western blot - Protective protein/Cathepsin A (PPCA) antibody (ab79590)
All lanes : Anti-Protective protein/Cathepsin A (PPCA) antibody (ab79590) at 1/500 dilution
Lane 1 : Extracts from Jurkat cells, treated with etoposide (25uM, 1hour) Lane 2 : Extracts from 293 cells, treated with etoposide (25uM, 1hour) Lane 3 : Extracts from 293 cells, treated with etoposide (25uM, 1hour) with Cl-peptide at 5 µg/ml
Lysates/proteins at 25 µg per lane.
Predicted band size : 32 , 54 kDa
References for Anti-Protective protein/Cathepsin A (PPCA) antibody (ab79590)
This product has been referenced in:
Lee C et al. NEU1 sialidase regulates the sialylation state of CD31 and disrupts CD31-driven capillary-like tube formation in human lung microvascular endothelia. J Biol Chem289:9121-35 (2014).
Read more (PubMed: 24550400) »