Anti-Progerin [13A4] | Ximbio 152141 product information

product summary

company name :

Ximbio

product type :

antibody

product name :

Anti-Progerin [13A4]

catalog :

152141

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

4000

reactivity :

human

application :

western blot, ELISA, immunoprecipitation

more info or order :

Ximbio product webpage

product information

Name :

Anti-Progerin [13A4]

Catalogue Number :

152141

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) :

Myeloma Used :

P3X63Ag8.653

Research Area :

Developmental Biology

Notes :

Does not recognise Lamin A or Lamin C.

more info or order :

Ximbio product webpage