Anti-Progerin Antibody, clone 13A4

Descrição detalhada : Progerin is a truncated version of lamin A protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a mutation (C1824T) in the lamin A gene, LMNA. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a deletion of 50 amino acids. 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. Researchers have shown that progerin activates genes that regulate stem cell differentiation via the Notch signaling pathway. Progerin, which has been linked to normal aging, is produced in healthy indivduals via "sporadic use of the cryptic splice site.
Sinónimos : LMNA, CMD1A, LMNC, LMN1, LDP1, LFP, FPLD, FPL, EMD2
Armazenamento : +2°C to +8°C
Embalagem : 1X1EA