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Lamin A + C R453W Antibody (12A-2F5) [Janelia Fluor® 646]

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Product Details

Summary
Reactivity HuSpecies Glossary
Applications WB, ELISA, ICC/IF, IP
Clone
12A-2F5
Clonality
Monoclonal
Host
Mouse
Conjugate
Janelia Fluor 646

Order Details

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Lamin A + C R453W Antibody (12A-2F5) [Janelia Fluor® 646] Summary

Immunogen
Synthetic peptide corresponding to amino acids 449-457 of human Lamin A + C [Accession#: NP_733821.1]
Specificity
Disease-associated point-mutant R453W of Lamin A + C
Isotype
IgG1 Kappa
Clonality
Monoclonal
Host
Mouse
Gene
LMNA
Purity
Protein G purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • ELISA
  • Immunocytochemistry/ Immunofluorescence
  • Immunoprecipitation
  • Western Blot
Application Notes
Optimal dilution of this antibody should be experimentally determined.

Packaging, Storage & Formulations

Storage
Store at 4C in the dark.
Buffer
50mM Sodium Borate
Preservative
0.05% Sodium Azide
Purity
Protein G purified

Notes

Sold under license from the Howard Hughes Medical Institute, Janelia Research Campus.

Alternate Names for Lamin A + C R453W Antibody (12A-2F5) [Janelia Fluor® 646]

  • 70 kDa Lamin
  • CDCD1
  • CDDC
  • Charcot Marie Tooth Disease Axonal Type 2B1
  • CMD1A
  • CMT2B1
  • EMD2
  • FPL
  • FPLD
  • HGPS
  • IDC
  • LAMIN A
  • Lamin A/C R453W
  • Lamin A/C
  • LAMIN C
  • LDP1
  • LFP
  • LGMD1B
  • LMN 1
  • LMN A
  • LMN C
  • LMN1
  • LMNA
  • LMNC
  • PRELAMIN A
  • PRO1
  • Progeria 1 (Hutchinson Gilford Type)

Background

The lamin A gene is important in making nuclear structure and substructures for DNA replication appropriately in cells. The R453W mutation of LMNA, however, negates much of these normal characteristics, and instead causes the phenotypes of Emery-Dreifuss muscular dystrophy (EDMD). The mutation impairs myoblast formation and differentiation, and contributes to muscle wasting. R453W lamin changes the location of the H2K27me3 modified histone interaction with chromatin, which inactivates certain downstream genes. It also affects the normal lamin function of scaffolding substrates for the MEK-ERK pathway, and disrupts the structure of other binding sites. Thus, R453W seems to cause phenotypes of muscular dystrophy both through direct genetic impact, but also through epigenetic influence over normal scaffolding functionality related to DNA replication.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Product General Protocols

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Video Protocols

WB Video Protocol
ICC/IF Video Protocol

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Secondary Antibodies

 

Isotype Controls

Additional Lamin A + C R453W Products

Array NBP1-77401JF646

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Bioinformatics

Gene Symbol LMNA