Cathepsin A, Recombinant Protein

Ctsa; Cathepsin A; AU019505; PPCA; Ppgb; lysosomal protective protein isoform b

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

E Coli
Source: Mouse

> 90%

Liquid
In Phosphate Buffered Saline (pH 7.4) containing 10% glycerol.

Store at -70 degree C for 12 months.
Centrifuge the vial prior to opening.

Small volumes of CTSA recombinant protein 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.

CTSA; also known as lysosomal protective protein isoform b; is a component of the lysosomal multienzyme complex along with beta-galactosidase and sialidase Neu1. It is a multicatalytic enzyme with deamidase and esterase in addition to carboxypeptidase activities. Recombinant mouse CTSA; fused to His-tag at C-terminus; was expressed in insect cell and purified by using conventional chromatography techniques.

SDS-PAGE

52.4 kDa

cathepsin A

lysosomal protective protein

Lysosomal protective protein

Ppgb; PPCA  [Similar Products]

This gene encodes a glycoprotein with deamidase, esterase and carboxypeptidase activities. The encoded protein associates with and provides a protective function to the lysosomal enzymes beta-galactosidase and neuraminidase. Deficiency of the related gene in humans results in galactosialidosis. The proprotein is processed into two shorter chains. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Jan 2013]

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; Endoplasmic reticulum; Mitochondrial; Protease

Chromosomal Location of Human Ortholog: 2 H3|2 85.27 cM

Cellular Component: intracellular membrane-bound organelle; membrane; mitochondrion; nucleoplasm

Molecular Function: protein binding; serine carboxypeptidase activity

Biological Process: proteolysis; proteolysis involved in cellular protein catabolic process; regulation of protein stability

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