艾杜糖-2-硫酸酯酶抗体

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英文名称  Anti-Iduronate 2 sulfatase

中文名称  艾杜糖-2-硫酸酯酶抗体

别    名  Alpha L iduronate sulfate sulfatase; Alpha-L-iduronate sulfate sulfatase; AW214631; Ids; IDS_HUMAN; Iduronate 2 sulfatase 14 kDa chain; Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate sulfatase; Idursulfase; MPS2; RP23-29M4.1; SIDS.
浓    度  1mg/1ml

规 格  0.2ml/200μg    

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Dog, Cow, Sheep

艾杜糖-2-硫酸酯酶抗体的多样性：

抗体的异质性。抗体的组成极为复杂，是由成千上万、多种多样的免疫球蛋白（Ig）分子所组成。这些Ig分子在形状、大小、结构以及氨基酸的组成和排列上，既相似，又有差别。由于有差别，它们的电泳活性就有很大的变化。

因为抗体具有与抗原决定簇相对应的结合部位（抗原结合簇），所以抗体与抗原的结合具有特异性。另一方面，抗体本身是一种蛋白质，具有本身的氨基酸组成、排列和立体结构，对异种动物来说，它又是抗原。各类Ig都具有可用血清学方法检出的抗原特异性，它们表现出不同的血清学类型。

艾杜糖-2-硫酸酯酶抗体的相关产品:

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Anti-β-arrestin 2β-抑制蛋白2抗体0.1ml

Anti-β-catenin/β-cats  β-连环蛋白抗体 0.1ml

产品类型  一抗    

研究领域  细胞生物 免疫学 细胞类型标志物  

蛋白分子量  predicted molecular weight: 47kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Iduronate 2 sulfatase 

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  ICC=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.  

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. 

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

产品介绍Subunit : Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a 42 kDa and a 14 kDa polypeptides.

Subcellular Location : Lysosome.

Tissue Specificity : Liver, kidney, lung, and placenta.

Post-translational modifications : The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity (By similarity).

DISEASE : Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.

艾杜糖-2-硫酸酯酶抗体Similarity : Belongs to the sulfatase family.

Database links : UniProtKB/Swiss-Prot: P22304.1