Anti-Protective protein抗体Lysosomal protective protein/cathepsin A （PPCA） is a lysosomal serine carboxypeptidase that forms an intralysosomal enzyme complex with

产品编号 yb-6040R
英文名称Anti-Protective protein抗体
中文名称 组织蛋白酶A抗体
别    名 Cathepsin A; CTSA; BETA GALACTOSIDASE PROTECTIVE PROTEIN; Carboxypeptidase C; Glactosialidosis; GLB2; Goldberg Syndrome; GSL; Lysosomal protective protein; NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE; NGBE; PPCA; PPGB; Protective protein for beta galactosidase; PPGB_HUMAN.
Anti-Protective protein抗体   
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 51kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Protective protein
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 background:
Lysosomal protective protein/cathepsin A (PPCA) is a lysosomal serine carboxypeptidase that forms an intralysosomal enzyme complex with ?galactosidase and neuraminidase (NEU1). PPCA is synthesized as a 54 kDa precursor/zymogen, and proteolytically cleaved in the lysosome into a catalytically active 32 and 20 kDa two chain enzyme. The enzyme has cathepsin A activity at acidic pH but maintains also a deamidase/esterase activity at neutral pH. Furthermore, the human enzyme, purified from plaets and lymphocytes, has been shown to function on the inactivation of selected neuropeptides, like substance P, oxytocin, and endothelin I. The autosomal recessive genetic deficiency of PPCA causes galactosialidosis, a neurodegenerative lysosomal storage disorder, resulting in the secondary deficiencies of ?galactosidase and NEU1.

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.

Subunit:
Heterodimer of a 32 kDa chain and a 20 kDa chain; disulfide-linked.

Subcellular Location:
Lysosome.

DISEASE:
Defects in CTSA are the cause of galactosialidosis (GSL) [MIM:256540]. 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.

Similarity:
Belongs to the peptidase S10 family.


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.