Progerin is expressed as the result of a de novo point mutation in the lamin A gene and is the underlying cause of Hutchison-Gilford progeria syndrome (accelerated aging). There is now also evidence that small amounts of Progerin are also produced in normal cells and that this might have a link with the normal aging process (Cao et al., PNAS, 2007 Mar 20;104(12):4949-54.). Gly608Gly is the most frequent HGPS-associated mutation. It is a silent base substitution that activates a cryptic splice donor in exon 11 of LMNA (BOX 3). Use of this anomalous splice donor leads to the loss of 150 nucleotides from the 3' end of exon 11 in the mature lamin A mRNA, and internal deletion of 50 amino-acid residues from the C terminus of lamin A. Progerin is the resulting mutant protein. Progerin retains its C-terminal CAAX motif, and therefore is farnesylated.

This antibody clone is manufactured by Abcam.

If you require this antibody in a particular buffer formulation or a particular conjugate for your experiments, please contact orders@abcam.com or you can find further information here.

Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

Please check that this product meets your needs before purchasing. If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, as well as customer reviews and Q&As.