Human Cathepsin A/CTSA Recombinant Protein (RPES4592)

SKU:
RPES4592
Product type:
Recombinant Protein
Host species:
Human Cells
Reactivity:
Human
€361

Description

ELISA Kit Technical Manual

Recombinant Human Cathepsin A/CTSA Protein (His Tag)

Background:Cathepsin A is active in cellular compartments called lysosomes. These compartments contain enzymes that digest and recycle materials when they are no longer needed. Cathepsin A interacts with the enzymes ?-galactosidase and neuraminidase 1, which play a role in the breakdown of complexes of sugar molecules (oligosaccharides) attached to certain proteins (glycoproteins) or fats (glycolipids). Cathepsin A forms a complex with these two enzymes and directs their transport within the cell to the lysosomes. Within lysosomes, cathepsin A activates the enzymes and prevents their breakdown.
Name:Recombinant Human Cathepsin A/CTSA Protein (His Tag)
Size:10µg
Assay Genie SKU:RPES4592
Synonyms:Lysosomal protective protein;CTSA;Carboxypeptidase C;Carboxypeptidase L;Cathepsin A;GLB2;GSL;NGBE;PPCA;PPGB
Species:Human
Expression host:Human Cells
Sequence:Ala29-Tyr480
Accession:P10619
Mol Mass:52.2 kDa
AP Mol Mass:58-60 kDa
Tag:C-6His
Purity:> 95 % as determined by reducing SDS-PAGE.
Endotoxin:< 1.0 EU per µg as determined by the LAL method.
Storage:Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.
Shipping:This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs.Upon receipt, store it immediately at<-20°C.
Formulation:Supplied as a 0.2 um filtered solution of 20mM PB,150mM NaCl,pH7.4.
Reconstitution:Please refer to the printed manual for detailed information.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Mitochondrial; EC 3.4.16.5; Protease; Endoplasmic reticulum

Chromosomal Location of Human Ortholog: 20q13.1

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

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

Biological Process: positive regulation of catalytic activity; intracellular protein transport; cellular protein metabolic process; sphingolipid 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

NCBI Summary: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]
UniProt Code:P10619
NCBI GenInfo Identifier:20178316
NCBI Gene ID:5476
NCBI Accession:P10619.2
UniProt Secondary Accession:P10619,Q561W6, Q5JZH1, Q96KJ2, Q9BR08, Q9BW68, B2R798
UniProt Related Accession:P10619
Molecular Weight:480
NCBI Full Name:Lysosomal protective protein
NCBI Synonym Full Names:cathepsin A
NCBI Official Symbol:CTSA
NCBI Official Synonym Symbols:GSL; GLB2; NGBE; PPCA; PPGB
NCBI Protein Information:lysosomal protective protein; deamidase; urinary kininase; carboxypeptidase C; carboxypeptidase L; carboxypeptidase-L; beta-galactosidase 2; lysosomal carboxypeptidase A; protective protein cathepsin A; carboxypeptidase Y-like kininase; beta-galactosidase protective protein
UniProt Protein Name:Lysosomal protective protein
UniProt Synonym Protein Names:Carboxypeptidase C; Carboxypeptidase L; Cathepsin A; Protective protein cathepsin A; PPCA; Protective protein for beta-galactosidaseCleaved into the following 2 chains:Lysosomal protective protein 32 kDa chain; Lysosomal protective protein 20 kDa chain
Protein Family:Lysosomal protective protein
UniProt Gene Name:CTSA
UniProt Entry Name:PPGB_HUMAN
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