Progerin Protein

Produktnummer ABIN2669338

Dieses Recombinant -Protein wird in Escherichia coli (E. coli) produziert.

Kurzübersicht für Progerin Protein (ABIN2669338)

Target: Progerin

Protein-Typ: Recombinant

Spezies: Ratte

Quelle: Escherichia coli (E. coli)

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

Reinheit: > 90 %

Produktdetails

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: 20 mM Tris-HCl pH 7.5, 0,3M NaCl, 1 mM DTT, 1,5 mM EDTA, 10 % (v/v) glycerol.

Lagerung: -20 °C

Antigendetails

Target: Progerin

Hintergrund: Progerin is a 614 amino acid 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.