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Anti-Cleaved-Cathepsin A Antibody

品牌：Antibodies

貨號：

規(guī)格：50μl

貨期：

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Anti-Cleaved-Cathepsin A Antibody

商品詳情參考文獻相關(guān)資料

Name:	Anti-Cleaved-Cathepsin A Antibody See all Cleaved-Cathepsin A primary antibodies
Description:	Rabbit polyclonal antibody to Cleaved-Cathepsin A
Specificity:	Cleaved-Cathepsin A pAb detects endogenous levels of Cleaved-Cathepsin A protein.
Applications:	WB, IHC, IF
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant full length Human Cleaved-Cathepsin A.
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Molecular Weight:	~ 32, 54 kDa
Purity:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form:	1mg/ml in PBS?with?0.1%?Sodium?Azide,?50%?Glycerol.

Function:	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.
Involvement in Disease:	Galactosialidosis: 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.
Sequence Similarities:	Belongs to the peptidase S10 family.
Cellular Location:	Lysosome.