Application

Monoclonal Anti-Strumpellin antibody has been used in immunoblotting immunoprecipitation flow cytometry

Biochem/physiol Actions

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).

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).

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General description

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.

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.

Immunogen

synthetic peptide corresponding to an internal region of human Strumpellin.

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.