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GNPTAB Rabbit pAb  Cite:0    分享
GNPTAB Rabbit pAb

货号： IM72058
复制产品信息
  • 种属：H，M，R，Dg，Pg，Cow，Sheep
  • 用途：WB，IF，ELISA，IHC-P，IF-ICC，IHC-F
规格:
50μl 100μl
单位:
支
单价:￥1350.00
产品简介
储存与保存
注意事项
产品简介
  • 货号
    IM72058
  • 产品名称
    GNPTAB Rabbit pAb
  • 别名
    GNPTA; ICD; EG432486; RGD1564821; GNPTA_HUMAN; GNPTAB; GlcNAc-1-phosphotransferase subunits alpha/beta; Stealth protein GNPTAB; UDP-N-acetylglucosamine-1-phosphotransferase subunits alpha/beta; 2.7.8.17; KIAA1208; GNPTA_MOUSE;
  • 抗体来源
    Rabbit
  • 克隆类型
    Polyclonal
  • 交叉反应
    predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Dog
  • 产品应用
    WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
  • 理论分子量
    143 kDa
  • 细胞定位
    细胞浆 细胞膜
  • 性状
    Liquid
  • 浓度
    1mg/ml
  • 免疫原
    KLH conjugated synthetic peptide derived from human N-acetylglucosamine-1-phosphotransferase subunit beta: 901-1000/1256
  • 亚型
    IgG
  • 纯化方法
    affinity purified by Protein A
  • 缓冲液
    0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
  • 产品介绍
    This gene encodes two of three subunit types of the membrane-bound enzyme N-acetylglucosamine-1-phosphotransferase, a heterohexameric complex composed of two alpha, two beta, and two gamma subunits. The encoded protein is proteolytically cleaved at the Lys928-Asp929 bond to yield mature alpha and beta polypeptides while the gamma subunits are the product of a distinct gene (GeneID 84572). In the Golgi apparatus, the heterohexameric complex catalyzes the first step in the synthesis of mannose 6-phosphate recognition markers on certain oligosaccharides of newly synthesized lysosomal enzymes. These recognition markers are essential for appropriate trafficking of lysosomal enzymes. Mutations in this gene have been associated with both mucolipidosis II and mucolipidosis IIIA.[provided by RefSeq, May 2010].
  • Function
    Catalyzes the formation of mannose 6-phosphate (M6P) markers on high mannose type oligosaccharides in the Golgi apparatus. M6P residues are required to bind to the M6P receptors (MPR), which mediate the vesicular transport of lysosomal enzymes to the endosomal/prelysosomal compartment.
  • Subunit
    Hexamer of two alpha, two beta and two gamma subunits; disulfide-linked. It is believed that the alpha and/or the beta subunit of the enzyme contain the catalytic portion and that the gamma subunit functions in recognition of the lysosomal enzymes.
  • Subcellular Location
    N-acetylglucosamine-1-phosphotransferase subunit alpha: Golgi apparatus membrane; Single-pass type I membrane protein. N-acetylglucosamine-1-phosphotransferase subunit beta: Golgi apparatus membrane; Single-pass type II membrane protein.
  • Tissue Specificity
    Expressed in the heart, whole brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • Post-translational modifications
    The alpha- and beta-subunits appear to be generated by a proteolytic cleavage at the Lys-928-Asp-929 bond.
  • DISEASE
    Defects in GNPTAB are the cause of mucolipidosis type II (MLII) [MIM:252500]; also known as inclusion cell disease or I-cell disease (ICD). MLII is a fatal, autosomal recessive, lysosomal storage disorder characterized by severe clinical and radiologic features, peculiar fibroblast inclusions, and no excessive mucopolysacchariduria. Congenital dislocation of the hip, thoracic deformities, hernia, and hyperplastic gums are evident soon after birth. Defects in GNPTAB are the cause of mucolipidosis type III complementation group A (MLIIIA) [MIM:252600]; also known as variant pseudo-Hurler polydystrophy. MLIIIA is an autosomal recessive disease of lysosomal enzyme targeting. Clinically MLIII is characterized by restricted joint mobility, skeletal dysplasia, and short stature. Mildly coarsened facial features and thickening of the skin have been described. Cardiac valvular disease and corneal clouding may also occur. Half of the reported patients show learning disabilities or mental retardation.
  • Similarity
    Belongs to the stealth family. Contains 1 EF-hand domain. Contains 2 LNR (Lin/Notch) repeats.
  • SWISS
    Q3T906
  • Gene ID
    79158
储存与保存

1.保存：-20℃
2.有效期：1年

注意事项

1.我司生产的生化试剂如无特殊标注，基本为非无菌包装，若用于细胞实验，请提前做好预处理。需低温保存的产品，一旦配成溶液，请分装保存，避免反复冻融造成的产品失效。

2.本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。 

3.为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。 

4.实验结果可由多种因素影响，相关处理只限于产品本身，不涉及其他赔偿。 

 

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