Rabbit Anti-delta Sarcoglycan/FITC Conjugated antibody
|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/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||心血管 细胞生物 细胞骨架 细胞外基质|
|交叉反应||Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||32kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human delta Sarcoglycan|
|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.|
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]
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.
Cell membrane > sarcolemma. Cytoplasm > cytoskelet
Most strongly expressed in skeletal and cardiac muscle. Also detected in smooth muscle. Weak expression in brain and lung.
Disulfide bonds are present.
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.
Belongs to the sarcoglycan beta/delta/gamma/zeta family.
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
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.