GLB2; GSL; NGBE; PPCA; PPGB

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen affinity purification

Store at -20 degree C. Avoid freeze / thaw cycles

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-CTSA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ELISA (EIA), Western Blot (WB)

WB: 1:200-1:1000

52,489 Da

cathepsin A

lysosomal protective protein; beta-galactosidase 2; beta-galactosidase protective protein; carboxypeptidase C; carboxypeptidase L; carboxypeptidase Y-like kininase; carboxypeptidase-L; deamidase; lysosomal carboxypeptidase A; protective protein cathepsin A; urinary kininase

Lysosomal protective protein

PPGB; PPCA??[Similar Products]

PPGB_HUMAN

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]

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/***** 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; intracellular membrane-bound organelle; membrane; 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; protein amino acid N-linked glycosylation via asparagine; glycosphingolipid metabolic process; post-translational protein modification; proteolysis

Disease: Galactosialidosis

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