产品封面图

Protective protein/Cathepsin A

antibody
收藏
  • ¥1380 - 2200
  • 康朗生物
  • kl-6040R
  • 中国/美国/德国
  • 2025年12月23日
  • WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500
  • Rabbit
  • Human, Mouse, Rat, Dog, Pig, Cow, Horse,
avatar
  • 企业认证

    点击 QQ 联系

  • 万千商家帮你免费找货

    0 人在求购买到急需产品
    • 详细信息
    • 文献和实验
    • 技术资料
    • 供应商

      上海康朗生物科技有限公司

    • 库存

      大量

    • 目录编号

      kl-6040R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      12个月

    • 抗体英文名

      Protective protein/Cathepsin A antibody

    • 抗体名

      组织蛋白酶A抗体

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse, Rat, Dog, Pig, Cow, Horse,

    • 免疫原

      KLH conjugated synthetic peptide derived from human Protective protein:401-480/480

    • 亚型

      IgG

    • 形态

      冻干粉或液体

    • 应用范围

      WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500

    • 浓度

      1mg/ml

    • 保存条件

      -20 °C

    • 规格

      100ul 200ul

    Protective protein/Cathepsin A antibody
    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.  
    规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    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=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Protective protein:401-480/480 
    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 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.

    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.

    SWISS:
    P10619

    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 产品细节图片1
    Sample: 
    Spleen (Mouse) Lysate at 40 ug
    Primary: Anti-Protective protein (bs-6040R) at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 51 kD
    Observed band size: 51 kD

    产品细节图片2
    Sample: 
    293T Cell (Human) Lysate at 30 ug
    Primary: Anti-Protective protein (bs-6040R) at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 51 kD
    Observed band size: 51 kD

    产品细节图片3
    Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Protective protein,Cathepsin A) Polyclonal Antibody, Unconjugated (bs-6040R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

    风险提示:丁香通仅作为第三方平台,为商家信息发布提供平台空间。用户咨询产品时请注意保护个人信息及财产安全,合理判断,谨慎选购商品,商家和用户对交易行为负责。对于医疗器械类产品,请先查证核实企业经营资质和医疗器械产品注册证情况。

    图标文献和实验
    相关实验
    • Antibody Conjugation

      of conjugated-Ig is added to the column. If 2mL of conjugated-Ig is added, discard first 1mL and keep the next 4mL. The antibody is always diluted by 2x after leaving the column. 5. When FITC coupling an antibody, you can determine the FITC to Protein ratio

    • Antibody Conjugation

      of conjugated-Ig is added to the column. If 2mL of conjugated-Ig is added, discard first 1mL and keep the next 4mL. The antibody is always diluted by 2x after leaving the column. 5. When FITC coupling an antibody, you can determine the FITC to Protein ratio

    • Antibody Labeling

      step 5 min Antibody 15 min PBS Wash 6 x 2 min Protein A Gold 15 min PBS Wash 6 x 2 min Water Wash 5 x 1 min. Blocking Any "sticky" protein can be used to cover non-specific binding sites on the thawed sections

    图标技术资料

    暂无技术资料 索取技术资料

    同类产品报价

    产品名称
    产品价格
    公司名称
    报价日期
    ¥1125
    攸碧艾(上海)生物技术有限公司
    2025年10月18日询价
    ¥880
    上海沪震实业有限公司
    2025年07月14日询价
    ¥2400
    深圳市豪地华拓生物科技有限公司
    2025年07月11日询价
    ¥590
    上海联迈生物工程有限公司
    2025年07月11日询价
    ¥220
    上海信裕生物科技有限公司
    2025年07月11日询价
    Protective protein/Cathepsin A antibody
    ¥1380 - 2200