05-1231 | Anti-Progerin Antibody, clone 13A4

05-1231
100 µg  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, ICC, ELISA, IPMPurifiedMonoclonal Antibody
      Description
      Catalogue Number05-1231
      Trade Name
      • Upstate
      DescriptionAnti-Progerin Antibody, clone 13A4
      Alternate Names
      • LMNA
      • CMD1A
      • LMNC
      • LMN1
      • LDP1
      • LFP
      • FPLD
      • FPL
      • EMD2
      Background InformationProgerin 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.
      References
      Product Information
      FormatPurified
      Control
      • HeLa cells expressing human progerin
      PresentationProtein G purified monoclonal antibody from ascites. Supplied at 1 mg/ml in 0.1 M Tris-Glycine (pH7.4), 150 mM NaCl, 0.05% sodium azide.
      Applications
      ApplicationThis Anti-Progerin Antibody, clone 13A4 is validated for use in WB, IC, ELISA, IP for the detection of Progerin.
      Key Applications
      • Western Blotting
      • Immunocytochemistry
      • ELISA
      • Immunoprecipitation
      Biological Information
      ImmunogenKLH-conjugated synthetic peptide corresponding to the amino acids 604-611 of progerin (lamin A/C).
      Clone13A4
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityRecognizes human progerin, Mr ~70 kDa. Does not recognize Lamin A or Lamin C
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Species Reactivity NoteHuman. Other species not tested.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Through alternate splicing, this gene encodes three type A lamin isoforms. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome. [provided by RefSeq].
      Gene Symbol
      • LMNA
      Purification MethodProtein G Purified
      UniProt Number
      Molecular Weight70 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceRoutinely evaluated by western blotting on HeLa cells expressing N-terminally Flag-tagged human progerin.
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      For maximum recovery of product, centrifuge the vial prior to removing the cap.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Progerin, clone 13A4 - 1956745 1956745
      Anti-Progerin, clone 13A4 - 2023193 2023193
      Anti-Progerin, clone 13A4 - 2198970 2198970
      Anti-Progerin, clone 13A4 - 2341109 2341109
      Anti-Progerin, clone 13A4 - 2499690 2499690
      Anti-Progerin, clone 13A4 - NG1532405 NG1532405
      Anti-Progerin, clone 13A4 - NG1848908 NG1848908
      Anti-Progerin, clone 13A4 -2719905 2719905
      Anti-Progerin, clone 13A4 -2723555 2723555
      Anti-Progerin, clone 13A4 -2780434 2780434

      References

      Reference overviewApplicationPub Med ID
      Progerin expression disrupts critical adult stem cell functions involved in tissue repair.
      Pacheco, LM; Gomez, LA; Dias, J; Ziebarth, NM; Howard, GA; Schiller, PC
      Aging  6  1049-63  2014

      Show Abstract
      Western Blotting25567453 25567453