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首頁>>免疫學>>一抗>>SGCE蛋白抗體
SGCE蛋白抗體
  • 產品貨號:
    BN40490R
  • 中文名稱:
    SGCE蛋白抗體
  • 英文名稱:
    Rabbit anti-SGCE Polyclonal antibody
  • 品牌:
    Biorigin
  • 貨號

    產品規格

    售價

    備注

  • BN40490R-100ul

    100ul

    ¥2360.00

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

  • BN40490R-200ul

    200ul

    ¥3490.00

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

產品描述

英文名稱SGCE
中文名稱SGCE蛋白抗體
別    名dystonia 11, myoclonic; DYT11; Epsilon sarcoglycan; Epsilon SG; Epsilon-sarcoglycan; Epsilon-SG; ESG; sarcoglycan, epsilon; sgcE; SGCE_HUMAN.  
研究領域細胞生物  發育生物學  信號轉導  細胞骨架  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用WB=1:500-2000 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50kDa
細胞定位細胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human SGCE:61-160/437 <Extracellular>
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This gene encodes the epsilon member of the sarcoglycan family. Sarcoglycans are transmembrane proteins that are components of the dystrophin-glycoprotein complex, which link the actin cytoskeleton to the extracellular matrix. Unlike other family members which are predominantly expressed in striated muscle, the epsilon sarcoglycan is more broadly expressed. Mutations in this gene are associated with myoclonus-dystonia syndrome. This gene is imprinted, with preferential expression from the paternal allele. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2010]

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 > cytoskeleton.

Tissue Specificity:
Ubiquitous.

DISEASE:
Defects in SGCE are a cause of dystonia type 11 (DYT11) [MIM:159900]; also known as myoclonic dystonia or alcohol-responsive dystonia. DYT11 is a myoclonic dystonia. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. DYT11 is characterized by involuntary lightning jerks and dystonic movements and postures alleviated by alcohol. Inheritance is autosomal dominant. The age of onset, pattern of body involvement, presence of myoclonus and response to alcohol are all variable.

Similarity:
Belongs to the sarcoglycan alpha/epsilon family.

SWISS:
O43556

Gene ID:
8910

Database links:

Entrez Gene: 407209 Cow

Entrez Gene: 8910 Human

Entrez Gene: 20392 Mouse

Entrez Gene: 100240725 Pig

Entrez Gene: 432360 Rat

Omim: 604149 Human

SwissProt: Q29S03 Cow

SwissProt: O43556 Human

SwissProt: O70258 Mouse

SwissProt: Q6YAT4 Rat

Unigene: 371199 Human

Unigene: 8739 Mouse

Unigene: 185815 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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