Anti-Lamin A/C LMNA Monoclonal Antibody BES2070LCA
货号 | BES2070LCA |
---|---|
产品名称 | Anti-Lamin A/C LMNA Monoclonal Antibody |
基因名 | LMNA |
抗体来源 | Mouse |
克隆 | Monoclonal |
抗体亚型 | Mouse |
分子量 | 74KD |
免疫原 | Full length recombinant human lamin A expressed in and purified from E. coli. |
内容 | Purified antibody at 1mg/mL in 50% PBS, 50% glycerol plus 5mM NaN3 |
浓度 | 500 ug/ml, actual concentration vary by lot. Use suggested dilution ratio to decide dilution procedure. |
保存条件 | 12 months from date of receipt,-20℃ as supplied. 6 months 2 to 8℃ after reconstitution. Avoid repeated freezing and thawing. |
背景资料 | Lamins are structural protein components of the nuclear lamina, a protein network underlying the inner nuclear membrane that determines nuclear shape and size. There are three types of lamins, A,B and C. The lamin A/C (LMNA) gene contains 12 exons. Alternative splicing within exon 10 gives rise to two different mRNAs that code for pre-lamin A and lamin C. Lamin A/C is mapped to 1q21.2-q21.3 and mutations in this gene cause a variety of human diseases including Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy, and Hutchinson-Gilford progeria syndrome. Lamin A/C deficiency is thus associated with both defective nuclear mechanics and impaired mechanically activated gene transcription. |
研究类别 | 1. Lammerding, J.; Schulze, P. C.; Takahashi, T.; Kozlov, S.; Sullivan, T.; Kamm, R. D.; Stewart, C. L.; Lee, R. T. : Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction. J. Clin. Invest. 113: 370-378, 2004.2. Lin, F.; Worman, H. J. : Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C. J. Biol. Chem. 268: 16321-16326, 1993.3. Wydner, K. L.; McNeil, J. A.; Lin, F.; Worman, H. J.; Lawrence, J. B. : Chromosomal assignment of human nuclear envelope protein genes LMNA, LMNB1, and LBR by fluorescence in situ hybridization. Genomics 32: 474-478, 1996. |
使用小鼠 mAb 对 lamin A/C 的不同细胞裂解物进行蛋白质印迹分析,M00438-1,绿色稀释 1:1,000:[1] 蛋白质标准(红色),[2] HeLa,[3] HEK293 [4] C6, [5] NIH-3T3 细胞裂解物。74 和 65kDa 的两个强条带分别对应于核纤层蛋白 A 和核纤层蛋白 C 蛋白,仅在人源细胞中检测到。抗体无法识别大鼠或小鼠蛋白质。