Lysosomal protective protein; Carboxypeptidase C; Carboxypeptidase L; Cathepsin A; Protective protein cathepsin A; PPCA; Protective protein for beta-galactosidase; Lysosomal protective protein 32 kDa chain; Lysosomal protective protein 20 kDa chain; CTSA; PPGB

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

Polyclonal

Rabbit Ig

Rabbit

This CTSA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 18-45 amino acids from the N-terminal region of human CTSA.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

CTSA 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.

Crown Antibodies; Metabolism; Signal Transduction

ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)

IHC~~1:50~100
WB~~1:1000

Immunohistochemical analysis of paraffin-embedded H. kidney section using CTSA Antibody (N-term). MBS9214956 was diluted at 1:25 dilution. A undiluted biotinylated goat polyvalent antibody was used as the secondary, followed by DAB staining.

Western blot analysis of lysates from A431, Hela, HepG2, HT-1080 cell line and human placenta, kidney tissue lysate (from left to right), using CTSA Antibody (N-term). MBS9214956 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 35ug per lane.

CTSA Antibody (N-term) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the CTSA antibody detected the CTSA protein (arrow).

CTSA Antibody (N-term) western blot analysis in mouse bladder tissue lysates (35ug/lane).This demonstrates the CTSA antibody detected the CTSA protein (arrow).

CTSA antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the CTSA antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.

54466

cathepsin A

lysosomal protective protein

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: Endoplasmic reticulum; Protease; EC 3.4.16.5; Mitochondrial

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

Reich, M., et al. Immunol. Lett. 128(2):143-147(2010)
Bonten, E.J., et al. J. Biol. Chem. 284(41):28430-28441(2009)
Ewing, R.M., et al. Mol. Syst. Biol. 3, 89 (2007) :
Tatano, Y., et al. J. Med. Invest. 53 (1-2), 103-112 (2006) :
Lewandrowski, U., et al. Mol. Cell Proteomics 5(2):226-233(2006)

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.