Progerin Protein

Catalog No. ABIN2669340

Recombinant protein expressed in Escherichia coli (E. coli).

Quick Overview for Progerin Protein (ABIN2669340)

Target: Progerin

Protein Type: Recombinant

Origin: Human

Source: Escherichia coli (E. coli)

Application: SDS-PAGE (SDS), Western Blotting (WB)

Purity: > 90 %

Product Details

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: 20 mM Tris-HCl pH 7.5, 0,5M NaCl, 1 mM DTT, 1,5 mM EDTA, 20 % (v/v) glycerol

Storage: -20 °C

Target Details

Target: Progerin

Background: Progerin is a 614 amino acids protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a point mutation (C1824T) in the LMNA gene that codes for lamin A and C. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a 50-amino acids internal deletion within the carboxyl-terminal domain of the protein. Approximately 80 % of Hutchinson-Gilford progeria syndrome cases carry a single copy of the most common mutation, a silent point mutation, G608G (GGC > GGT), within exon 11 of LMNA gene. Lamin A constitutes a major structural component of the lamina, a scaffold of proteins found inside the nuclear membrane of a cell, progerin does not properly integrate into the lamina, which disrupts the scaffold structure and leads to significant disfigurement of the nucleus, characterized by a lobular shape.