骨骼肌烯醇化酶抗体

规格：1mg/1ml

英文名： ENO3

别名： 2 phospho D glycerate hydrol yase; 2 phospho D glycerate hydrolyase; Beta enolase; ENO3; Enolase 3 (beta muscle); Enolase 3; Enolase3; GSD13; MSE; Muscle specific enolase; Skeletal muscle enolase; ENO

分子量： 47kDa

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

免疫原：KLH conjugated synthetic peptide derived from human ENO3

交叉反应：Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Sheep,

细胞定位：

骨骼肌烯醇化酶抗体产品介绍：background: ENO3 is also known as beta enolase, one of the three enolase isoenzymes found in mammals. A switch from alpha enolase to beta enolase occurs in muscle tissue during development and ENO3, a homodimer, is found in skeletal muscle cells in the adult and appears to have a function in striated muscle development and regeneration. Mutations can result in decreased stability of the enzyme and be associated with a glycogen storage myopathy. This results in exercise-induced myalgias, generalized muscle weakness and fatigability. Function: Appears to have a function in striated muscle development and regeneration. Subunit: Mammalian enolase is composed of 3 isozyme subunits, alpha, beta and gamma, which can form homodimers or heterodimers which are cell-type and development-specific. Interacts with PNKD. Subcellular Location: Cytoplasm. Note=Localized to the Z line. Some colocalization with CKM at M-band (By similarity). Tissue Specificity: 骨骼肌烯醇化酶抗体The alpha/alpha homodimer is expressed in embryo and in most adult tissues. The alpha/beta heterodimer and the beta/beta homodimer are found in striated muscle, and the alpha/gamma heterodimer and the gamma/gamma homodimer in neurons. DISEASE: Glycogen storage disease 13 (GSD13) [MIM:612932]: A metabolic disorder that results in exercise-induced myalgias, generalized muscle weakness and fatigability. It is characterized by increased serum creatine kinase and decreased enolase 3 activity. Dramatically reduced protein levels with focal sarcoplasmic 骨骼肌烯醇化酶抗体accumulation of glycogen-beta particles are detected on ultrastructural analysis. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the enolase family. Database links: UniProtKB/Swiss-Prot: P13929.5 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

骨骼肌烯醇化酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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：肿瘤  细胞生物  免疫学  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid