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    IDUA Rabbit pAb (bs-15542R)  
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    產(chǎn)品編號 bs-15542R
    英文名稱(chēng) IDUA Rabbit pAb
    中文名稱(chēng) α-L-艾杜糖苷酶抗體
    別    名 IDUA_HUMAN; alpha-L-iduronidase; IDA; Iduronidase alpha L; MPS1.  
    研究領(lǐng)域 腫瘤  細胞生物  免疫學(xué)  信號轉導  細胞類(lèi)型標志物  細胞骨架  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human,Mouse
    產(chǎn)品應用 WB=1:500-2000,ELISA=1:5000-10000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 70 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human IDUA 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 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].

    Subunit:
    Monomer (Probable).

    Subcellular Location:
    Lysosome.

    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.

    SWISS:
    P35475

    Gene ID:
    3425

    Database links:

    Entrez Gene: 3425 Human

    Entrez Gene: 15932 Mouse

    Omim: 252800 Human

    SwissProt: P35475 Human

    SwissProt: P48441 Mouse

    Unigene: 89560 Human



    產(chǎn)品圖片
    Sample: Lane 1: Mouse Kidney tissue lysates Lane 2: Mouse Lung tissue lysates Lane 3: Human U87MG cell lysates Lane 4: Human HeLa cell lysates Lane 5: Human A549 cell lysates Primary: Anti-IDUA (bs-15542R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 70 kDa Observed band size: 70 kDa
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