Monoclonal Anti-Lamin A/C R482W (mouse IgG1 isotype) is derived from the hybridoma 5H8-B4 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a recombinant protein. Lamin A and lamin C are members of the A-type lamins. A-type lamins contain an α-helical rod domain, a nuclear localization sequence, and a carboxy-terminal CAAX box. Lamin A is a structural protein of the nuclear lamina. The LMNA gene is located on the human chromosome at 1q22.
Monoclonal Anti-Lamin A/C R482W recognizes a human Lamin A/C recombinant protein containing the R482W substitution.
recombinant protein fragment containing the R482W substitution of human Lamin A/C.1 The isotype is determined by ELISA using Mouse Monoclonal Antibody Isotyping Reagents (Sigma ISO-2).
Monoclonal Anti-Lamin A/C R482W antibody produced in mouse may be used in:
Mutations in Lamin A and C are associated with a variety of rare human diseases including muscular, cardiomyopathy, lipodystrophy, dystrophy, neuropathy and progeroid syndromes collectively termed laminopathies. It is also associated with premature aging, Hutchinson-Gilford progeria syndrome. Most diseases arise from dominant, missense mutations.
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
Storage and Stability
For continuous use, store at 2-8°C for up to one month. For extended storage, freeze at -20oC in working aliquots. Repeated freezing and thawing,or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.