Human Lysosomal protective protein ELISA Kit | CTSA elisa kit

Human Lysosomal protective protein ELISA Kit

Cat. Num. AAA9427648

• Gene Names: CTSA; GSL; GLB2; NGBE; PPCA; PPGB
• Reactivity: Human
• Synonyms: Lysosomal protective protein; Human Lysosomal protective protein ELISA Kit; Carboxypeptidase C; Carboxypeptidase L; Cathepsin A; Protective protein cathepsin A; PPCA; Protective protein for beta-galactosidase; CTSA; PPGB; 3.4.16.5; CTSA elisa kit

• Reactivity: Human

• Samples: Serum, plasma, tissue homogenates and other biological fluids.
• Detection Range: 78-5000 pg/mL
• Sensitivity: Less than 33pg/mL
• Assay Time: 3-5h
• Sample Volume: 100T
• Detection Wavelength: 450 nm
• Preparation and Storage: Store at 4 degree C

NCBI and Uniprot Product Information

• NCBI GI #: 119395729
• NCBI GeneID: 5476
• NCBI Accession #: NP_000299.2
• NCBI GenBank Nucleotide #: NM_000308.3
• UniProt Accession #: P10619; Q561W6; Q5JZH1; Q96KJ2; Q9BR08; Q9BW68; B2R798
• Molecular Weight: 52,489 Da
• NCBI Official Full Name: lysosomal protective protein isoform a
• NCBI Official Synonym Full Names: cathepsin A
• NCBI Official Symbol: CTSA
• NCBI Official Synonym Symbols: GSL; GLB2; NGBE; PPCA; PPGB
• NCBI Protein Information: lysosomal protective protein
• UniProt Protein Name: Lysosomal protective protein
• Protein Family: Lysosomal protective protein
• UniProt Gene Name: CTSA
• UniProt Synonym Gene Names: PPGB; PPCA

NCBI Description

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]

Uniprot Description

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/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 3.4.16.5; Endoplasmic reticulum; Mitochondrial; Protease

Chromosomal Location of Human Ortholog: 20q13.12

Cellular Component: endoplasmic reticulum; extracellular region; 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; neutrophil degranulation; proteolysis; proteolysis involved in cellular protein catabolic process; regulation of protein stability

Disease: Galactosialidosis