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Rabbit Anti-delta Sarcoglycan  antibody (bs-14264R)  
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產(chǎn)品編號 bs-14264R
英文名稱 Rabbit Anti-delta Sarcoglycan  antibody
中文名稱 肌營養(yǎng)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.  
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,Rabbit,Pig,Sheep,Cow)
產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 32kDa
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human delta Sarcoglycan: 51-150/289 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



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