Protective protein/Cathepsin A抗体特异性结合抗原：抗体本身不能直接溶解或杀伤带有特异抗原的靶细胞，通常需要补体或吞噬细胞等共同发挥效应以**病原微生物或导致病理损伤。然而，抗体可通过与病毒或**的特异性结合，直接发挥中和病毒的作用。
产品编号xy- 6040R
英文名称Protective protein/Cathepsin A
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100ul 200ul
研究领域细胞生物 **学
抗体来源Rabbit
克隆类型Polyclonal
Protective protein/Cathepsin A抗体交叉反应 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/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.
Protective protein/Cathepsin A抗体保存条件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.
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.
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/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
Protective protein/Cathepsin A抗体（antibody,
Ab）是由效应B细胞（效应**B细胞）分泌，机体用于抵御外来物质，如病毒，**等抗原，结构呈“Y”字型的球状蛋白质，仅仅存在于脊椎动物的血液和B**细胞膜表面。凡是能够跟抗体结合的物质，均被称作抗原，因此对于抗抗体（能够结合抗体的抗体）来说，抗体本身也是一种抗原物质。
Protective protein/Cathepsin A抗体普通抗体重链和轻链的结构
重链结构：普通的**球蛋白具有2条重链（H链），分子量约为50kD,有μ、δ、γ、ε和α五种重链亚型，对应的**球蛋白名称分别为IgM、IgG、IgA、IgD和IgE。
轻链结构： 普通**球蛋白具有2条轻链（L链），分子质量约25kDa，有κ链和λ链两种亚型，这两种轻链决定了Ig的亚型类别（IgG1，IgG2，IgG3，IgG4）。一个天然的Ig分子两条轻链总是相同的，但在同一个体内可存在分别带有κ或λ链的抗体分子。不同种属生物体内两型轻链的比例不同，正常人血清**球蛋白κ链：λ链约为2：1，而在小鼠的比例为20：1。
2.2抗体Fab段和Fc段
IgG经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1个Fc段,每个Fab段都为单价,可与抗原结合但不会再发生凝集反应；经胃蛋白酶酶切后裂解为1个完整F(ab)2片段和碎片化的Fc片段,F(ab’)2片段为双价,可同时结合两个抗原表位。Fab段为抗原结合片段（fragment of antigen binding，Fab），相当于抗体分子的两个臂，由一个完整的轻链和重链的VH和CH1结构域组成。Fc段为可结晶段（fragment crystallizable，Fc）相当于Ig的CH2和CH3结构域，是Ig与效应分子或者细胞相互作用的部位。Fab段包含完整的可变区，以及恒定区的CH1区域。Fc段仅指Ig恒定区CH2和CH3的区域，相当于Y字结构下面那一部分。
合格 合格 HMGCS1 三羟基**基辅酶A合成酶1
合格 LASS5 长寿相关基因lASS5抗体
合格 TNFSF15 肿瘤坏死因子配体超家族成员15抗体
合格 TECK 胸腺表达趋化因子抗体
合格 IGFBP7 胰岛素样生长因子结合蛋白7抗体
合格 WISP2 结缔组织生长因子样蛋白抗体
合格 CLIC1 氯离子通道蛋白27抗体
合格 Cystatin A 胱抑素A/半胱氨酸蛋白酶抑制剂A抗体
合格 Hsc70 热休克蛋白71抗体
合格 合格 TUBA1C 微管相关蛋白α6抗体
合格 Laminin B2 gamma 1 层粘连蛋白B2γ1抗体
合格 ITLN1 内皮细胞凝集素HL1抗体
合格 ALDH1B1 乙醛脱氢酶5抗体
合格 BAG2 Bcl-2结合抑凋亡蛋白2抗体
合格 CREM 环磷酸腺苷反应元件调节蛋白抗体
合格 合格 TFEB T**细胞转录调节因子TFEB抗体
合格 TAF12 TATA框结合蛋白关联因子12抗体
合格 DP1 转录因子DP1抗体
合格 合格 AKT3 蛋白激酶B3
合格 合格 Cdc7 细胞分裂周期蛋白7抗体
合格 phospho-LSP1 (Ser252) 磷酸化白细胞F肌动蛋白结合蛋白抗体
合格 FHL2 相互作用蛋白FHL2抗体
合格 MYLPF 快速骨骼肌肌球蛋白轻链2抗体
合格 phospho-Amyloid Precursor Protein (Ser730) 磷酸化APP淀粉样肽前体蛋白抗体