肌营养蛋白δ/δ-sarcoglycan抗体
规格：1mg/1ml
英文名： delta Sarcoglycan
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; MGC
分子量： 32kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human delta Sa
交叉反应：Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
细胞定位：细胞膜
肌营养蛋白δ/δ-sarcoglycan抗体产品介绍：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 > 肌营养蛋白δ/δ-sarcoglycan抗体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肌营养蛋白δ/δ-sarcoglycan抗体 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. 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.
肌营养蛋白δ/δ-sarcoglycan抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：心血管 细胞生物 细胞骨架 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid