Progerin mouse antibody (mAb) (Clone 13A4), sample

Progerin is a 614 amino acid protein related to the Lamin A protein and is involved in Hutchinson-Gilford progeria syndrome (HGPS), a rare genetic disorder characterized by features reminiscent of severe premature aging. Progerin is most often generated by a point mutation in the gene that codes for lamin A and C. Nuclear lamins are intermediate filament proteins that are the major structural component of the nuclear lamina on the inner surface of the nuclear envelope. Progerin lacks a conserved proteolytic cleavage site and therefore retains its C-terminus and is permanently farnesylated. Although it can enter the nucleus and associate with the nuclear envelope, it cannot incorporate normally into the nuclear lamina. HGPS is caused by the toxic accumulation of Progerin, which acts to deregulate mitosis and DNA damage signaling, leading to premature senescence and cell death.