SAB4200647
Monoclonal Anti-Strumpellin antibody produced in mouse
clone St-1, purified from hybridoma cell culture
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100 μL
$530.00
About This Item
UNSPSC Code:
12352203
NACRES:
NA.41
biological source
mouse
Quality Level
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
St-1, monoclonal
form
buffered aqueous solution
mol wt
antigen ~120 kDa
species reactivity
mouse, rat, human
concentration
~1 mg/mL
technique(s)
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.
isotype
IgG1
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... KIAA0196(9897)
General description
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.
Immunogen
synthetic peptide corresponding to an internal region of human Strumpellin.
Application
Monoclonal Anti-Strumpellin antibody has been used in
- immunoblotting
- immunoprecipitation
- flow cytometry
Biochem/physiol Actions
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).
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
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.
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Storage Class
10 - Combustible liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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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
Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases
Clemen C S, et al.
Brain, 133(10), 2920-2941 (2010)
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