Anti-Alpha Dystroglycan抗体(ab103905)

概述

  • 产品名称Anti-Alpha Dystroglycan抗体
    参阅全部 Alpha Dystroglycan 一抗
  • 描述
    山羊多克隆抗体to Alpha Dystroglycan
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
    预测可用于: Rabbit, Cow, Dog
  • 免疫原

    Synthetic peptide corresponding to Human Alpha Dystroglycan aa 569-582 (internal sequence).
    Sequence:

    C-HVGKHEYFMHATDK

  • 阳性对照
    • Human Skeletal Muscle lysates.
  • 常规说明Reported variants represent identical protein: NP_001159400.1, NP_004384.3.

性能

应用

Our Abpromise guarantee covers the use of ab103905 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 97 kDa.

靶标

  • 功能The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization.
    Alpha-dystroglycan is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains, and for certain adenoviruses. Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. Also acts as a receptor for M.leprae in peripheral nerve Schwann cells but only in the presence of the G-domain of LAMA2, and for lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses.
    Beta-dystroglycan is a transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.
  • 组织特异性Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane. Also expressed in keratinocytes and fibroblasts.
  • 疾病相关Defects in DAG1 are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C7 (MDDGC7) [MIM:613818]. An autosomal recessive muscular dystrophy showing onset in early childhood, and associated with mental retardation without structural brain anomalies. Note=MDDGC7 is caused by DAG1 mutations that interfere with normal post-translational processing, resulting in defective DAG1 glycosylation and impaired interactions with extracellular-matrix components. Other muscular dystrophy-dystroglycanopathies are caused by defects in enzymes involved in protein O-glycosylation.
  • 序列相似性Contains 1 peptidase S72 domain.
  • 翻译后修饰O- and N-glycosylated. Alpha-dystroglycan is heavily O-glycosylated comprising of up to two thirds of its mass and the carbohydrate composition differs depending on tissue type. Mucin-type O-glycosylation is important for ligand binding activity. O-mannosylation of alpha-DAG1 is found in high abundance in both brain and muscle where the most abundant glycan is Sia-alpha-2-3-Gal-beta-1-4-Glc-NAc-beta-1-2-Man. In muscle, glycosylation on Thr-379 by a phosphorylated O-mannosyl glycan with the structure 2-(N-acetylamido)-2-deoxygalactosyl-beta-1,3-2-(N-acetylamido)-2-deoxyglucosyl-beta-1,4-6-phosphomannose is mediated by like-acetylglucosaminyltransferase (LARGE) protein and is required for laminin binding. O-mannosylation is also required for binding lymphocytic choriomeningitis virus, Old World Lassa fever virus, and clade C New World arenaviruses. The O-glycosyl hexose on Thr-367, Thr-369, Thr-372, Thr-381 and Thr-388 is probably mannose. O-glycosylated in the N-terminal region with a core 1 or possibly core 8 glycan. The beta subunit is N-glycosylated.
    Autolytic cleavage produces the alpha and beta subunits. In cutaneous cells, as well as in certain pathological conditions, shedding of beta-dystroglcan can occur releasing a peptide of about 30 kDa.
    SRC-mediated phosphorylation of the PPXY motif of the beta subunit recruits SH2 domain-containing proteins, but inhibits binding to WWW domain-containing proteins, DMD and UTRN. This phosphorylation also inhibits nuclear entry.
  • 细胞定位Secreted > extracellular space and Cell membrane. Cytoplasm > cytoskeleton. Nucleus > nucleoplasm. The monomeric form translocates to the nucleus via the action of importins and depends on RAN. Nuclear transport is inhibited by Tyr-892 phosphorylation. In skeletal muscle, this phosphorylated form locates to a vesicular internal membrane compartment. In peripheral nerves, localizes to the Schwann cell membrane. Colocalizes with ERM proteins in Schwann-cell microvilli.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 156DAG antibody
    • A3a antibody
    • AGRNR antibody
    • Alpha dystroglycan antibody
    • Alpha-DG antibody
    • Beta-DG antibody
    • Beta-dystroglycan antibody
    • DAG antibody
    • Dag1 antibody
    • DAG1_HUMAN antibody
    • Dystroglycan 1 (dystrophin associated glycoprotein 1) antibody
    • Dystroglycan antibody
    • Dystrophin associated glycoprotein 1 antibody
    • Dystrophin-associated glycoprotein 1 antibody
    • OTTHUMP00000210857 antibody
    • OTTHUMP00000210858 antibody
    see all

Anti-Alpha Dystroglycan antibody 图像

  • Anti-Alpha Dystroglycan antibody (ab103905) at 0.2 µg/ml + Human Skeletal Muscle lysate at 35 µg

    Predicted band size : 97 kDa
  • All lanes : Anti-Alpha Dystroglycan antibody (ab103905) at 0.1 µg/ml

    Lane 1 : Mouse Skeletal Muscule Lysate in RIPA buffer
    Lane 2 : Rat Skeletal Muscule Lysate in RIPA buffer

    Lysates/proteins at 35 µg per lane.

    Developed using the ECL technique

    Predicted band size : 97 kDa

Anti-Alpha Dystroglycan antibody (ab103905)参考文献

ab103905 has not yet been referenced specifically in any publications.

Product Wall

Application Western blot
Sample Zebrafish Tissue lysate - whole (Muscle)
Gel Running Conditions Reduced Denaturing (6% Acrylamide)
Loading amount 15 µg
Specification Muscle
Blocking step Milk as blocking agent for 3 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 20°C
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提交于 Apr 10 2017

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