生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
分子量
127 kDa
种属反应性
mouse, rat, human
浓度
1 mg/mL
技术
ELISA: suitable
western blot: suitable
NCBI登记号
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... KIAA0196(9897)
一般描述
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
Antibody was raised against a 19 amino acid peptide near the carboxy terminus of human Strumpellin.
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).
特点和优势
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联系
The action of this antibody can be blocked using blocking peptide SBP3500640.
外形
Supplied at 1 mg/mL in PBS with 0.02% sodium azide.
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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
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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