Lamin A + C R482W Antibody (5H8-B4)

Images

 
Western Blot: Lamin A + C R482W Antibody (5H8-B4) [NBP1-77402] - Analysis of Lamin A + C R482W in HeLa cells transfected with Flag-tagged Lamin A 1) wild type, 2) R482W and 3) R453W.

Product Details

Summary
Reactivity HuSpecies Glossary
Applications WB, ELISA, ICC/IF, IP
Clone
5H8-B4
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated

Order Details

Lamin A + C R482W Antibody (5H8-B4) Summary

Immunogen
Hepatitis B Virus Capsid Antigen (HBcAg) Lamin A + C Ig-fold R482W fusion protein.
Specificity
Disease-associated point-mutant R482W of Lamin A + C
Isotype
IgG1 Kappa
Clonality
Monoclonal
Host
Mouse
Gene
LMNA
Purity
Unpurified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Western Blot 1:1000
  • ELISA 1:100-1:2000
  • Immunocytochemistry/Immunofluorescence 1:10-1:2000
  • Immunoprecipitation 1:10-1:500
Application Notes
This Lamin A + C R482W (5H8-B4) point-mutant specific antibody is useful for Western blot, ELISA, Immunoprecipitation and Immunocytochemistry/Immunofluorescence. In WB bands can be seen at 74kDa (Lamin A) and 65 kDa (Lamin C).
Publications
Read Publication using
NBP1-77402 in the following applications:

  • 1 publication
  • IP
    1 publication
  • WB
    1 publication

Reactivity Notes

Human

Packaging, Storage & Formulations

Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
Ascites
Preservative
0.1% Sodium Azide
Purity
Unpurified

Alternate Names for Lamin A + C R482W Antibody (5H8-B4)

  • 70 kDa Lamin
  • CDCD1
  • CDDC
  • Charcot Marie Tooth Disease Axonal Type 2B1
  • CMD1A
  • CMT2B1
  • EMD2
  • FPL
  • FPLD
  • HGPS
  • IDC
  • LAMIN A
  • Lamin A/C R82W
  • 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

In eukaryotic organisms, lamin A-type proteins i.e. lamin A/C are major components of lamina, a thin proteinaceous filamentous meshwork that underlies the inner nuclear membrane and besides regulating nuclear shape/rigidity, it also provide a scaffold-type support for chromosomes or replicating DNA to interact with epigenetic machinery. Lamin A/C gene (LMNA) mutations leads to neuromuscular and cardiac dystrophies, lipodystrophies, and premature ageing syndromes. In LMNA gene, missense mutations affecting two residues located within Ig-fold domain are common in laminopathy patients (i) an exchange of arginine 453 by tryptophan (R453W) detected in 16% of autosomal-dominant EDMD cases (11% laminopathy cases), (ii) the substitution of arginine 482 by either tryptophan, glutamine, or leucine (R482W/Q/L), which is responsible for more than 80% of FPLD (familial partial lipodystrophy - 13% laminopathy cases). FPLD is a disorder which results in abnormal fat distribution, insulin resistance, dyslipidemia and early cardiac heart disease. Our Lamin A+C R482W (5H8-B4) is a novel monoclonal antibody that specifically detects disease causing point mutants of A-type lamins namely FPLD associated lamin A/C R482W mutant (does not cross-react with wild-type counterpart) and was generated by immunization with a fusion protein consisting of the hepatitis B virus capsid protein, HBcAg, and the lamin A/C Ig-fold R482W domain inserted at the highly immunogenic viral c/e1 epitope. This antibody has been validated by Western blotting, competition experiments, IP, and ICC/IF microscopy assays, can be applied to selectively study features of the mutant A-type lamins in primary patient cells.

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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Publications for Lamin A + C R482W Antibody (NBP1-77402)(1)

We have publications tested in 1 confirmed species: Human.

We have publications tested in 3 applications: ICC/IF, IP, WB.


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ICC/IF
(1)
IP
(1)
WB
(1)
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Video Protocols

WB Video Protocol
ICC/IF Video Protocol

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Secondary Antibodies

 

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Lamin A + C R482W NBP1-77402

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Bioinformatics

Gene Symbol LMNA
Entrez
Uniprot