Lamin A/C (LMNA, LMNC, CMD1A, HGPS, LGMD1B, LMN1, LMNL1, MADA, PRO1)

Size

£626.00

SKU:: 052238
Purity:: Purified by immunoaffinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Partial recombinant protein corresponding to human Lamin A/C at C-terminal.
Format:: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide, 50% glycerol.
Extra Details:: Lamins A and C are structural components of the lamina, a scaffold of proteins localized to the nuclear inner membrane, and contribute to nuclear membrane dynamics and chromatin structure. Mutations in the LMNA gene have been linked to multiple laminopathy disorders, including Hutchinson-Gilford progeria syndrome (HGPS), an autosomal dominant disorder characterized by the appearance of accelerated and dramatic aging. Patients with HGPS have a mutation in the LMNA gene that results in aberrant splicing and production of the toxic, truncated Lamin A protein progerin that functions in a dominant negative pattern. Applications: Suitable for use in Western Blot and Immunocytochemistry. Other applications not tested. Recommended Dilutions: Immunocytochemistry: 1-5ug/ml Western Blot: 10ul/5ml Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.