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Lysosomal protective protein

Below are the list of possible Lysosomal protective protein products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.

Lysosomal protective protein

 Lysosomal protective protein ELISA Kit
 Lysosomal protective protein Recombinant
 Lysosomal protective protein Antibody
Also known as Lysosomal protective protein (Carboxypeptidase C) (Carboxypeptidase L) (Cathepsin A) (Protective protein cathepsin A) (PPCA) (Protective protein for beta-galactosidase).
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 a
ctivity. 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; Endoplasmic reticulum; Mitochondrial; Protease

Chromosomal Location of Human Ortholog: 20q13.1

Cellular Component: endoplasmic reticulum; intracellular membrane-bound organelle; lysosomal lumen; membrane; nucleoplasm

Molecular Function: carboxypeptidase activity; enzyme activator activity; exo-alpha-sialidase activity; serine carboxypeptidase activity

Biological Process: glycosphingolipid metabolic process; intracellular protein transport; proteolysis; proteolysis involved in cellular protein catabolic process; regulation of protein stability

Disease: Galactosialidosis
 CTSA Recombinant
 CTSA Antibody
 PPGB Recombinant
 PPGB Antibody
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