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  • 产品名称:PrelaminAProtein

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PrelaminAProtein
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Characteristics Recombinant prelamin A is a 74 kDa precursor of the nuclear lamin A protein
Purity > 90 %
Background Nuclear lamin A is a structural component of the nuclear lamina encoded by lamin A/C gene (LMNA). Due to the presence of a CAAX box sequence at carboxyl terminus, prelamin A in vivo undergoes to serial post-translational modifications, resulting in the farnesylation of the cysteine thiol, removal of the AAX tripeptide, carboxyl-methylation of the cysteinyl carboxyl group and proteolysis of 18 C-terminal amino acids residues that lead to mature lamin A. Different mutations in the LMNA gene have been shown to be associated with different diseases 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.
Application Notes Optimal working dilution should be determined by the investigator.
Restrictions For Research Use only
Format Liquid
Buffer 20 mM phosphate buffer pH 7.0, 0.5M NaCl, 1 mM DTT, 1.5 mM EDTA, 20 % (v/v) glycerol
Storage -20 °C
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