SGCE蛋白抗体
规格：1mg/1ml
英文名： SGCE
别名： dystonia 11, myoclonic; DYT11; Epsilon sarcoglycan; Epsilon SG; Epsilon-sarcoglycan; Epsilon-SG; ESG; sarcoglycan, epsilon; sgcE; SGCE_HUMAN.
分子量： 50kDa
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 SGCE
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, Guinea Pig,
细胞定位：细胞膜
SGCE蛋白抗体产品介绍：background: 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 SGCSGCE蛋白抗体E 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. SGCE蛋白抗体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. Database links: Entrez Gene: 407209 Cow Entrez Gene: 8910 Human Entrez Gene: 20392 Mouse Entrez Gene: 100240725 Pig Entrez Gene: 432360 Rat Entrez Gene: 368230 Zebrafish 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.
SGCE蛋白抗体产品应用：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：细胞生物 发育生物学 信号转导 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit