SAB4200422

Sigma-Aldrich

Anti-Lamin A/C R482W antibody, Mouse monoclonal

clone 5H8-B4, purified from hybridoma cell culture

Synonym(s):
Monoclonal Anti-LMN1, Monoclonal Anti-LMNA, Monoclonal Anti-Prelamin-A/C, Monoclonal Anti-Lamin A/C R482W antibody produced in mouse, Monoclonal Anti-Lamin A/C
NACRES:
NA.41
Pricing and availability is not currently available.

Quality Level

biological source

mouse

antibody form

purified from hybridoma cell culture

antibody product type

primary antibodies

clone

5H8-B4, monoclonal

form

buffered aqueous solution

species reactivity

human

concentration

~1.0 mg/mL

application(s)

western blot: 0.1-0.2 μg/mL using a recombinant protein encoding a fragment of human Lamin A/C containing the R482W 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 R482W (mouse IgG1 isotype) is derived from the hybridoma 5H8-B4 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a recombinant protein. Lamin A and lamin C are members of the A-type lamins. A-type lamins contain 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 located on the human chromosome at 1q22.

Specificity

Monoclonal Anti-Lamin A/C R482W recognizes a human Lamin A/C recombinant protein containing the R482W substitution.

Immunogen

recombinant protein fragment containing the R482W substitution of human Lamin A/C.1 The isotype is determined by ELISA using Mouse Monoclonal Antibody Isotyping Reagents (Sigma ISO-2).

Application

Monoclonal Anti-Lamin A/C R482W antibody produced in mouse may be used in:
  • immunoblotting
  • immunoprecipitation
  • immunofluorescence

Biochem/physiol Actions

Mutations in Lamin A and C are associated with a variety of rare human diseases including muscular, cardiomyopathy, lipodystrophy, dystrophy, neuropathy and progeroid syndromes collectively termed laminopathies. It is also associated with 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
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...
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)
Jian-Hua Chen et al.
Disease models & mechanisms, 10(12), 1411-1420 (2017-10-07)
Adipose tissue is the primary tissue affected in most single gene forms of severe insulin resistance, and growing evidence has implicated it as a site at which many risk alleles for insulin resistance identified in population-wide studies might exert their...
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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