CTSA (Lysosomal protective protein)/GLB2/PPCA/GSL/NGBE/PPGB/Lysosomal Carboxypeptidase A/beta-galactosidase 2/beta-galactosidase protective protein/Carboxypeptidase C/Carboxypeptidase L/cathepsin A/protective protein for beta-galactosidase (galactosialidosis)/Protective protein cathepsin A/Protective protein for beta-galactosidase

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

Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of CTSA elisa kit 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.

MBS764996 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Lysosomal protective protein (CTSA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing CTSA. The ELISA analytical biochemical technique of the MBS764996 kit is based on CTSA antibody-CTSA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect CTSA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, CTSA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-CTSA antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-CTSA antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the CTSA amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of CTSA can be calculated.

52,489 Da

cathepsin A

lysosomal protective protein

Lysosomal protective protein

PPGB; PPCA??[Similar Products]

PPGB_HUMAN

This gene encodes a member of the peptidase S10 family of serine carboxypeptidases. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate two chains that comprise the heterodimeric active enzyme. This enzyme possesses deamidase, esterase and carboxypeptidase activities and acts as a scaffold in the lysosomal multienzyme complex. Mutations in this gene are associated with galactosialidosis. [provided by RefSeq, Nov 2015]

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; Protease; Mitochondrial

Chromosomal Location of Human Ortholog: 20q13.1

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

Molecular Function: carboxypeptidase activity; enzyme activator activity; serine carboxypeptidase activity

Biological Process: cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; glycosphingolipid metabolic process; intracellular protein transport; positive regulation of catalytic activity; post-translational protein modification; protein amino acid N-linked glycosylation via asparagine; proteolysis; proteolysis involved in cellular protein catabolic process; regulation of protein stability; sphingolipid metabolic process

Disease: Galactosialidosis

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