Progerin antibody (mAb)

for Progerin antibody (mAb) (Clone 13A4)Background

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. The mutation of the lamin A gene that leads to preogerin results in the creation of a cryptic splice site in lamin A mRNA, resulting in the deletion of 50 amino acids in the C-terminal region the lamin A protein. The 50 amino acid deletion removes a conserved proteolytic cleavage site and therefore Progerin retains its C-terminal 15 amino acids 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.

for Progerin antibody (mAb) (Clone 13A4)Immunogen

This Progerin antibody was raised against a peptide corresponding to amino acids 604-611 of human Progerin.

for Progerin antibody (mAb) (Clone 13A4)Application Notes

This Progerin antibody has been validated for use in immunofluorescence (2 - 5 μg/ml), immunoprecipitation (2 - 10 μg per IP) and Western blotting (1 - 2 μg/ml). See references for further details.

for Progerin antibody (mAb) (Clone 13A4)Buffer

Purified IgG in 70 mM Tris (pH 8), 105 mM NaCl, 32 mM glycine, 0.07 mM EDTA, 30% glycerol, and 0.035% sodium azide.

for Progerin antibody (mAb) (Clone 13A4)References

This antibody has been used in the following references:
IF: Capanni C et al (2010) Cell Cycle 9: 2600-2610.
WB: Capanni C et al (2010) Cell Cycle 9: 2600-2610.

for Progerin antibody (mAb) (Clone 13A4)Storage

Antibodies in solution can be stored at -20°C for 2 years. Avoid repeated freeze/thaw cycles and keep on ice when not in storage.

for Progerin antibody (mAb) (Clone 13A4)Guarantee

This product is for research use only and is not for use in diagnostic procedures. This product is guaranteed for 6 months from date of arrival.