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Synthetic peptide corresponding to Human Progerin (C terminal) conjugated to Keyhole Limpet Haemocyanin (KLH).
This antibody is available as an azide-free product (see ab180465).
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.
Our Abpromise guarantee covers the use of ab66587 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000. Detects a band of approximately 70 kDa.|
|ELISA||Use at an assay dependent dilution.|
|IP||Use at an assay dependent dilution. use 20ul/assay|