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Anti-Progerin [13A4]

Invented by Egon Ogris at Medical University Of Vienna

Info

Applications ELISA IP WB
Antigen/Gene or Protein Targets Progerin
Reactivity Human
Relevance 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 signalling pathway. Progerin, which has been linked to normal ageing, is produced in healthy individuals via "sporadic use of the cryptic splice site.
Host Mouse
Immunogen KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of Progerin
Subclass IgG1 kappa
Positive Control HeLa cells expressing human progerin
Molecular Weight (kDa) 70
Myeloma Used P3X63Ag8.653
Notes Does not recognise Lamin A or Lamin C.
Research Area Developmental Biology

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