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Products Polyclonal Anti-Lamin A/C
| Catalogue number: | PA1103 |
| Price: | $200.00 |
| Reactivities: | Human, Mouse, Rat |
| Applications: | Immunocytochemistry, Immunohistochemistry, Western Blot |
| Size: | 100ug/vial |
| Gene: | LMNA |
| Swiss prot: | P02545 |
| Form: | Lyophilized |
| Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
| Storage temp: | "At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing. " |
| Scientific background: | 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 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. |
| References: | 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. |
| Additional info: | A synthetic peptide corresponding to a sequence at the C-terminal of human Lamin A/C, identical to the related rat and mouse sequences. |
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