Protective protein,组织蛋白酶A抗体

产品编号HZ-6040R
英文名称Protective protein
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.
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,
Protective protein,组织蛋白酶A抗体产品应用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.
Protective protein,组织蛋白酶A抗体PubMedPubMed
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 platelets 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.

Protective protein,组织蛋白酶A抗体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.

Gene ID:
5476

Database links:


Entrez Gene: 5476 Human
Entrez Gene: 19025 Mouse
Omim: 613111 Human
SwissProt: P10619 Human
SwissProt: P16675 Mouse
Unigene: 609336 Human
Unigene: 359633 Mouse
Unigene: 474586 Mouse

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

产品图片

Tissue/cell: rat liver tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Protective protein/ Cathepsin A Polyclonal Antibody, Unconjugated(HZ-6040R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining