α-L-艾杜糖苷酶抗体

以下为您提供的是α-L-艾杜糖苷酶抗体的说明书,了解更多关于α-L-艾杜糖苷酶抗体的英文名称,α-L-艾杜糖苷酶抗体别名,α-L-艾杜糖苷酶抗体规格,请看以下详细介绍.

英文名称  Anti-IDUA

中文名称  α-L-艾杜糖苷酶抗体

别    名  IDUA_HUMAN; Alpha-L-iduronidase; IDA; Iduronidase alpha L; MPS1.

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, Horse

α-L-艾杜糖苷酶抗体的多样性：

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。

α-L-艾杜糖苷酶抗体的相关产品:

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Anti-γ-Adaptin衔接蛋白γ抗体0.1ml

Anti-κOR   kappa型阿片受体抗体 1ml

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产品类型  一抗    

研究领域  肿瘤 细胞生物 免疫学 信号转导 细胞类型标志物 细胞骨架  

蛋白分子量  predicted molecular weight: 70kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human IDUA

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）

亚    型  IgG

 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 This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). [provided by RefSeq, Jul 2008].

Tissue Specificity : Ubiquitous.

DISEASE : Mucopolysaccharidosis 1H (MPS1H) [MIM:607014]: A severe form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1H usually present, within the first year of life, a combination of hepatosplenomegaly, skeletal deformities, corneal clouding and severe mental retardation. Obstructive airways disease, respiratory infection and cardiac complications usually result in death before 10 years of age. Note=The disease is caused by mutations affecting the gene represented in this entry.

Mucopolysaccharidosis 1H/S (MPS1H/S) [MIM:607015]: A form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. MPS1H/S represents an intermediate phenotype of the MPS1 clinical spectrum. It is characterized by relatively little neurological involvement, but most of the somatic symptoms described for severe MPS1 develop in the early to mid-teens, causing considerable loss of mobility. Note=The disease is caused by mutations affecting the gene represented in this entry.

Mucopolysaccharidosis 1S (MPS1S) [MIM:607016]: A mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints stiffness, mild hepatosplenomegaly, aortic valve disease and corneal clouding. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity : Belongs to the glycosyl hydrolase 39 family.

Database links : UniProtKB/Swiss-Prot: P35475.2

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

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 α-L-艾杜糖苷酶抗体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.

Similarity : Belongs to the sulfatase family.

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