General description
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.
The previously assigned protein identifier Q6UYC3 has been merged into P02545. Full details can be found on the UniProt database.
Specificity
Other species not tested.
Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
Immunogen
KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).
Application
Research Category
Cell Structure
This Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin.
Quality
Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin.
Target description
70 kDa
Physical form
Format: Purified
Protein G Purified
Protein G purified immunoglobulin. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide.
Storage and Stability
Stable for 1 year at 2-8°C from date of receipt.
For maximum recovery of product, centrifuge the vial prior to removing the cap.
Analysis Note
Control
HeLa cells expressing human progerin
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Shipping Information:
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- UPC:
- 51131648
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- MPN:
- 05-1231
- Temperature Control Device:
- Yes
