Cat#:RP-5200H;Product Name:Recombinant Human Prelamin-A Protein;Synonym:Prelamin-A/C, LMNA, LMN1, FPL, IDC, LFP, CDDC, EMD2, FPLD, HGPS, LDP1, LMNC, PRO1, CDCD1, CMD1A, FPLD2, LMNL1, CMT2B1, LGMD1B.;Description:Recombinant Prelamin-A is a 74kDa precursor of the nuclear lamin A protein. Prelamin-A is a structural component of the nuclear lamina and it is encoded by lamin A/C gene (LMNA). Due to the presence of a CAAX box sequence at carboxyl terminus, Prelamin-A in vivo goes through a serial of post-translational modifications, resulting in the farnesylation of the cysteine thiol, removal of the AAX tripeptide, carboxyl-methylation of the cysteinyl carboxy group and proteolysis of 18 C-terminal amino acids residues that lead to mature lamin A. Diverse mutations in the lamin A/C gene are associated with different deseases that are collectively called laminophaties, including Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome.?Recombinant human prelamin A is fused to a 6 Histidine tag at the N-terminus.;Source:E.coli;AA Sequence:metpsqrrat rsgaqasstp lsptritrlq ekedlqelnd rlavyidrvr sletenaglr lriteseevv srevsgikaa yeaelgdark tldsvakera rlqlelskvr eefkelkarn tkkegdliaa qarlkdleal lnskeaalst alsekrtleg elhdlrgqva kleaalgeak kqlqdemlrr vdaenrlqtm keeldfqkni yseelretkr rhetrlveid ng;Purity:Greater than 90% as determined by SDS-PAGE.;Formulation:The Prelamin-A solution (0.1mg/ml) contains 10% Glycerol.;Stability:Recombinant Proteins are stable for up to 1 year from date of receipt at -70℃;Storage:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid repeated freeze-thaw cycles.;