delta Sarcoglycan肌营养蛋白δ/δ-sarcoglycan抗体

delta Sarcoglycan肌营养蛋白δ/δ-sarc

oglycan抗体
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  • ¥1380 - 2200
  • LMAI Bio
  • LM-14264R
  • 进口/国产
  • 2025年07月15日
  • WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
  • Rabbit
  • Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, 
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    • 详细信息
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 目录编号

      LM-14264R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      1年

    • 抗体英文名

      delta Sarcoglycan

    • 抗体名

      肌营养蛋白δ/δ-sarcoglycan抗体

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, 

    • 免疫原

      KLH conjugated synthetic peptide derived from human delta Sarcoglycan:51-150/289 

    • 亚型

      IgG

    • 形态

      Lyophilized or Liquid

    • 应用范围

      WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)

    • 浓度

      1mg/ml

    • 保存条件

      Store at -20 °C

    • 规格

      100ul  200ul

    delta Sarcoglycan肌营养蛋白δ/δ-sarcoglycan抗体
    英文名称 delta Sarcoglycan
    中文名称 肌营养蛋白δ/δ-sarcoglycan抗体
    别    名 35 kDa dystrophin associated glycoprotein; 35 kDa dystrophin-associated glycoprotein; 35DAG; CMD1L; DAGD; Delta-sarcoglycan; Delta-SG; Dystrophin associated glycoprotein delta sarcoglycan; LGMD2F; MGC22567; Placental delta sarcoglycan; Sarcoglycan delta (35 kDa dystrophin associated glycoprotein); SG delta; SGCD; SGCD_HUMAN; SGCDP; SGD.  
    规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    研究领域 心血管  细胞生物  细胞骨架  细胞外基质  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, 
    产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 32kDa
    细胞定位 细胞膜 
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human delta Sarcoglycan:51-150/289 
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    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.
    PubMed PubMed
    产品介绍 background:
    The protein encoded by this gene is one of the four known components of the sarcoglycan complex, which is a subcomplex of the dystrophin-glycoprotein complex (DGC). DGC forms a link between the F-actin cytoskeleton and the extracellular matrix. This protein is expressed most abundantly in skeletal and cardiac muscle. Mutations in this gene have been associated with autosomal recessive limb-girdle muscular dystrophy and dilated cardiomyopathy. Alternatively spliced transcript variants encoding distinct isoforms have been observed for this gene. [provided by RefSeq, Jul 2008]

    Function:
    Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.

    Subcellular Location:
    Cell membrane > sarcolemma. Cytoplasm > cytoskelet

    Tissue Specificity:
    Most strongly expressed in skeletal and cardiac muscle. Also detected in smooth muscle. Weak expression in brain and lung.

    Post-translational modifications:
    Glycosylated.
    Disulfide bonds are present.

    DISEASE:
    Defects in SGCD are the cause of limb-girdle muscular dystrophy type 2F (LGMD2F) [MIM:601287]. LGMD2F is an autosomal recessive disorder.
    Defects in SGCD are the cause of cardiomyopathy dilated type 1L (CMD1L) [MIM:606685]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

    Similarity:
    Belongs to the sarcoglycan beta/delta/gamma/zeta family.

    SWISS:
    Q92629

    Gene ID:
    6444

    Database links:

    Entrez Gene: 6444 Human

    Entrez Gene: 24052 Mouse

    Omim: 601411 Human

    SwissProt: Q92629 Human

    SwissProt: P82347 Mouse

    Unigene: 387207 Human

    Unigene: 644733 Human

    Unigene: 338890 Mouse



    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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