NP_001161066.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
52,489 Da
NCBI Official Full Name
lysosomal protective protein isoform c
NCBI Official Synonym Full Names
cathepsin A
NCBI Protein Information
lysosomal protective protein; deamidase; urinary kininase; carboxypeptidase C; carboxypeptidase L; carboxypeptidase-L; beta-galactosidase 2; lysosomal carboxypeptidase A; protective protein cathepsin A; carboxypeptidase Y-like kininase; beta-galactosidase protective protein
UniProt Protein Name
Lysosomal protective protein
UniProt Synonym Protein Names
Carboxypeptidase C; Carboxypeptidase L; Cathepsin A; Protective protein cathepsin A; PPCA; Protective protein for beta-galactosidaseCleaved into the following 2 chains:Lysosomal protective protein 32 kDa chain; Lysosomal protective protein 20 kDa chain
UniProt Synonym Gene Names
UniProt Entry Name
PPGB_HUMAN
NCBI Summary for CTSA
This gene encodes a glycoprotein which associates with lysosomal enzymes beta-galactosidase and neuraminidase to form a complex of high molecular weight multimers. The formation of this complex provides a protective role for stability and activity. Deficiencies in this gene are linked to multiple forms of galactosialidosis. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Comments for CTSA
CTSA: Protective 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. Defects in CTSA are the cause of galactosialidosis (GSL). 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. Belongs to the peptidase S10 family.
Protein type: EC 3.4.16.5; Protease; Mitochondrial; Endoplasmic reticulum
Chromosomal Location of Human Ortholog: 20q13.1
Cellular Component: nucleoplasm; lysosomal lumen; membrane; intracellular membrane-bound organelle; endoplasmic reticulum; lysosome
Molecular Function: serine carboxypeptidase activity; carboxypeptidase activity; enzyme activator activity
Biological Process: positive regulation of catalytic activity; intracellular protein transport; sphingolipid metabolic process; cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; glycosphingolipid metabolic process; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification; proteolysis
Disease: Galactosialidosis
Research Articles on CTSA
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Products associated with CTSA elisa kit
Pathways associated with CTSA elisa kit
Diseases associated with CTSA elisa kit
Organs/Tissues associated with CTSA elisa kit
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