SAB4200421

Sigma-Aldrich

Anti-Lamin A/C R453W antibody, Mouse monoclonal

clone 12A-2F5, purified from hybridoma cell culture

Synonym(s):
Anti-LMN1, Anti-LMNA, Anti-Pre lamin-A/C, Monoclonal Anti-Lamin A/C R453W antibody produced in mouse
NACRES:
NA.41

Quality Level

biological source

mouse

antibody form

purified from hybridoma cell culture

antibody product type

primary antibodies

clone

12A-2F5, monoclonal

form

buffered aqueous solution

mol wt

antigen ~47 kDa

species reactivity

human

concentration

~1.0 mg/mL

application(s)

western blot: 0.2-0.5 μg/mL using human Lamin A/C partial recombinant protein containing the R453W substitution

isotype

IgG1

conjugate

unconjugated

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

Gene Information

human ... LMNA(4000)

General description

Monoclonal Anti-Lamin A/C R453W (mouse IgG1 isotype) is derived from the hybridoma 12A-2F5 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a synthetic peptide. Lamin A and lamin C belong to the A-type lamins. A-type lamins are characterized by an α-helical rod domain, a nuclear localization sequence, and a carboxy-terminal CAAX box. Lamin A is a structural protein of the nuclear lamina. The LMNA gene is mapped on the human chromosome at 1q22.

Specificity

Monoclonal Anti-Lamin A/C R453W recognizes a human Lamin A/C recombinant protein containing the R453W substitution. This antibody does not recognize the Lamin A/C wild-type protein.

Immunogen

synthetic peptide containing the R453W substitution of human Lamin A/C

Application

Monoclonal Anti-Lamin A/C R453W antibody produced in mouse may be used in:
  • immunoblotting
  • immunoprecipitation
  • immunofluorescence

Biochem/physiol Actions

Mutations in Lamin A and C have been linked to a variety of rare human diseases including muscular dystrophy, lipodystrophy, cardiomyopathy, neuropathy and progeroid syndromes collectively termed laminopathies and to premature aging, Hutchinson-Gilford progeria syndrome. Most diseases arise from dominant, missense mutations.

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

Storage and Stability

For continuous use, store at 2-8°C for up to one month. For extended storage, freeze at -20oC in working aliquots. Repeated freezing and thawing,or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.

Disclaimer

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.

RIDADR

NONH for all modes of transport

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificate of Analysis

Certificate of Origin

Vienna E Brunt et al.
The Journal of physiology, 596(20), 4831-4845 (2018-08-18)
Accumulating evidence indicates that passive heat therapy (chronic use of hot tubs or saunas) has widespread physiological benefits, including enhanced resistance against novel stressors ('stress resistance'). Using a cell culture model to isolate the key stimuli that are likely to...
Evaluation of a Congenital Infantile Fibrosarcoma by Comprehensive Genomic Profiling Reveals an LMNA-NTRK1 Gene Fusion Responsive to Crizotinib.
Victor Wong et al.
Journal of the National Cancer Institute, 108(1) (2015-11-14)
Howard J Worman et al.
The Journal of clinical investigation, 113(3), 349-351 (2004-02-03)
Mutations in lamins A and C, nuclear intermediate-filament proteins in nearly all somatic cells, cause a variety of diseases that primarily affect striated muscle, adipocytes, or peripheral nerves or cause features of premature aging. Two new studies (see the related...
L Rao et al.
The Journal of cell biology, 135(6 Pt 1), 1441-1455 (1996-12-01)
Expression of the adenovirus E1A oncogene stimulates both cell proliferation and p53-dependent apoptosis in rodent cells. p53 implements apoptosis in all or in part through transcriptional activation of bax, the product of which promotes cell death. The adenovirus E1B 19K...
Nicola Carboni et al.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 32(1), 7-17 (2013-07-16)
Mutations on the LMNA gene are responsible for an heterogeneous group of diseases. Overlapping syndromes related to LMNA gene alterations have been extensively reported. Study scope is to perform a systematic analysis of the overlapping syndromes so far described and...

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