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Rabbit Anti-delta Sarcoglycan/APC Conjugated antibody (bs-14264R-APC)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-14264R-APC
英文名稱 Rabbit Anti-delta Sarcoglycan/APC Conjugated antibody
中文名稱 APC標記的肌營養蛋白δ/δ-sarcoglycan抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  細胞骨架  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human delta Sarcoglycan
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 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.
產品介紹 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.

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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