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    β1,4-N乙酰半乳糖氨基轉移酶1抗體
    • 產品貨號:
      BN40386R
    • 中文名稱:
      β1,4-N乙酰半乳糖氨基轉移酶1抗體
    • 英文名稱:
      Rabbit anti-B4GALNT1/GM2 synthase Polyclonal antibody
    • 品牌:
      Biorigin
    • 貨號

      產品規格

      售價

      備注

    • BN40386R-100ul

      100ul

      ¥2360.00

      交叉反應:Human,Mouse,Rat(predicted:Dog,Cow,Horse,Rabbit,Sheep) 推薦應用:WB,IHC-P,IHC-F,ICC,IF,ELISA

    • BN40386R-200ul

      200ul

      ¥3490.00

      交叉反應:Human,Mouse,Rat(predicted:Dog,Cow,Horse,Rabbit,Sheep) 推薦應用:WB,IHC-P,IHC-F,ICC,IF,ELISA

    產品描述

    英文名稱B4GALNT1/GM2 synthase
    中文名稱β1,4-N乙酰半乳糖氨基轉移酶1抗體
    別    名(N acetylneuraminyl) galactosylglucosylceramide; (N-acetylneuraminyl)-galactosylglucosylceramide; 4 N-acetylgalactosaminyltransferase 1; 4933429D13Rik; B4GALNT1; B4GN1_HUMAN; Beta 1 4 N acetyl galactosaminyl transferase 1; Beta 1 4 N acetylgalactosaminyltransferase 1; Beta-1; BETA-1,4-N-ACETYLGALACTOSAMINYLTRANSFERASE 1; BETA-1,4-N-ACETYLGALACTOSAMINYLTRANSFERASE; Beta1 4GalNAc T; Gal-NAc-T; GALGT; Galgt1; GalNAc T; GalNAc-T; GALNACT; GD2 synthase; Ggm-2; Ggm2; GM2 synthase; GM2/GD2 synthase; SIAT 2; SIAT2; UDP Gal:betaGlcNAc beta 1 4 N acetylgalactosaminyltransferase transferase 1; UDP N acetyl alpha D galactosamine:(N acetylneuraminyl).  
    研究領域細胞生物  神經生物學  信號轉導  轉錄調節因子  新陳代謝  
    抗體來源Rabbit
    克隆類型Polyclonal
    交叉反應Mouse,  (predicted: Human, Rat, Dog, Cow, Horse, Rabbit, Sheep, )
    產品應用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復)
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量59kDa
    細胞定位細胞漿 細胞膜 細胞外基質 
    性    狀Liquid
    濃    度1mg/ml
    免 疫 原KLH conjugated synthetic peptide derived from human B4GALNT1/GM2 synthase:451-533/533 
    亞    型IgG
    純化方法affinity purified by Protein A
    儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
    PubMedPubMed
    產品介紹GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. GalNAc-T is the enzyme involved in the biosynthesis of G(M2) and G(D2) glycosphingolipids. GalNAc-T catalyzes the transfer of GalNAc into G(M3) and G(D3) by a beta-1,4 linkage, resulting in the synthesis of G(M2) and G(D2), respectively. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2013]

    Function:
    Involved in the biosynthesis of gangliosides GM2, GD2 and GA2.

    Subunit:
    Homodimer; disulfide-linked.

    Subcellular Location:
    Golgi apparatus membrane; Single-pass type II membrane protein.

    DISEASE:
    Spastic paraplegia 26, autosomal recessive (SPG26) [MIM:609195]: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG26 is a complicated form characterized by onset in the first 2 decades of life of gait abnormalities due to lower limb spasticity and muscle weakness. Some patients have upper limb involvement. Additional features include intellectual disability, peripheral neuropathy, dysarthria, cerebellar signs, extrapyramidal signs, and cortical atrophy. The disorder is slowly progressive. Note=The disease is caused by mutations affecting the gene represented in this entry.

    Similarity:
    Belongs to the glycosyltransferase 2 family.

    SWISS:
    Q00973

    Gene ID:
    2583

    Database links:

    Entrez Gene: 2583 Human

    Entrez Gene: 14421 Mouse

    Entrez Gene: 64828 Rat

    Omim: 601873 Human

    SwissProt: Q00973 Human

    SwissProt: Q09200 Mouse

    SwissProt: Q10468 Rat

    Unigene: 159481 Human

    Unigene: 386762 Mouse

    Unigene: 10119 Rat



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


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