生物来源
mouse
质量水平
抗体形式
purified immunoglobulin
抗体产品类型
primary antibodies
克隆
St-1, monoclonal
表单
buffered aqueous solution
分子量
antigen ~120 kDa
种属反应性
mouse, rat, human
浓度
~1 mg/mL
技术
flow cytometry: 10-20 μg/test using using HeLa cells.
immunoblotting: 2.5-5 μg/mL
immunoprecipitation (IP): 5-10 μg using using lysates of rat A10 cells.
同位素/亚型
IgG1
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... KIAA0196(9897)
相关类别
一般描述
Monoclonal Anti-Strumpellin (mouse IgG1 isotype) is derived from the hybridoma St-1 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a synthetic peptide corresponding to an internal region of human Strumpellin, conjugated to KLH.
The gene KIAA0196 (strumpellin) is mapped to human chromosome 8q24. It is a widely expressed gene and the protein localizes in the cytoplasm and endoplasmic reticulum.
免疫原
synthetic peptide corresponding to an internal region of human Strumpellin.
应用
Monoclonal Anti-Strumpellin antibody has been used in
- immunoblotting
- immunoprecipitation
- flow cytometry
生化/生理作用
KIAA0196 (strumpellin) is involved in the WASH (WASP and Scar homologue) complex, an actin-regulating complex. It works as an interlink between actin regulation and endosomal membrane dynamics. Mutations in KIAA0196 are associated with spastic paraplegia and RSS (Ritscher-Schinzel syndrome).
Strumpellin, causes hereditary spastic paraplegia, a progressive neurodegenerative disorder clinically characterized by central motor system deficits leading to spastic paraparesis of the lower limbs. Strumpellin presence was observed in protein aggregate diseases affecting striated muscles and the Central nervous system (CNS).
外形
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
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
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
常规特殊物品
Exome sequencing expands the mutational spectrum of SPG8 in a family with spasticity responsive to L-DOPA treatment.
Conceição Bettencourt et al.
Journal of neurology, 260(9), 2414-2416 (2013-07-25)
A novel KIAA0196 (SPG8) mutation in a Chinese family with spastic paraplegia.
Xianling Wang et al.
Chinese medical journal, 127(10), 1987-1989 (2014-05-16)
Alison M Elliott et al.
Journal of medical genetics, 50(12), 819-822 (2013-09-26)
Ritscher-Schinzel syndrome (RSS) is a clinically heterogeneous disorder characterised by distinctive craniofacial features in addition to cerebellar and cardiac anomalies. It has been described in different populations and is presumed to follow autosomal recessive inheritance. In an effort to identify
Caroline Freeman et al.
Biochimica et biophysica acta, 1832(1), 160-173 (2012-10-23)
Mutations in the gene encoding strumpellin cause autosomal dominant hereditary spastic paraplegia (HSP), in which there is degeneration of corticospinal tract axons. Strumpellin is a component of the WASH complex, an actin-regulating complex that is recruited to endosomes by interactions
Christoph S Clemen et al.
Brain : a journal of neurology, 133(10), 2920-2941 (2010-09-14)
Mutations of the human valosin-containing protein gene cause autosomal-dominant inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia. We identified strumpellin as a novel valosin-containing protein binding partner. Strumpellin mutations have been shown to cause hereditary spastic
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