Anti-Progerin Antibody, clone 13A4

产品说明

一般描述

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.

特异性

Recognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
Other species not tested.

免疫原

KLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).

应用

Research Category
Cell Structure
This Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin.

质量

Routinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin.

目标描述

70 kDa

外形

Format: 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.
Protein G Purified

储存及稳定性

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.

分析说明

Control
HeLa cells expressing human progerin

其他说明

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

法律信息

UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany

免责声明

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.

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