SAB4200422
Anti-Lamin A/C R482W antibody, Mouse monoclonal
clone 5H8-B4, purified from hybridoma cell culture
别名:
Monoclonal Anti-LMN1, Monoclonal Anti-LMNA, Monoclonal Anti-Lamin A/C, Monoclonal Anti-Lamin A/C R482W antibody produced in mouse, Monoclonal Anti-Prelamin-A/C
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所有图片(1)
About This Item
UNSPSC代码:
12352203
NACRES:
NA.41
推荐产品
生物来源
mouse
偶联物
unconjugated
抗体形式
purified from hybridoma cell culture
抗体产品类型
primary antibodies
克隆
5H8-B4, monoclonal
表单
buffered aqueous solution
种属反应性
human
浓度
~1.0 mg/mL
技术
western blot: 0.1-0.2 μg/mL using a recombinant protein encoding a fragment of human Lamin A/C containing the R482W substitution.
同位素/亚型
IgG1
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... LMNA(4000)
一般描述
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.
特异性
Monoclonal Anti-Lamin A/C R482W recognizes a human Lamin A/C recombinant protein containing the R482W substitution.
免疫原
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).
应用
Monoclonal Anti-Lamin A/C R482W antibody produced in mouse may be used in:
- immunoblotting
- immunoprecipitation
- immunofluorescence
生化/生理作用
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.
外形
0.01M 磷酸缓冲盐溶液，pH 7.4，含 15mM 叠氮化钠。
储存及稳定性
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.
免责声明
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.
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储存分类代码
10 - Combustible liquids
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
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
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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