Strumpellin protein is encoded by Wiskott−Aldrich syndrome protein and scar homolog complex subunit 5 (WASHC5) gene. It is located on the human chromosome at 8q24.13. It is a component of the WASH complex.
Specificity
Anti-Strumpellin recognizes human, rat and mouse Strumpellin.
Immunogen
synthetic peptide corresponding to an internal region of human Strumpellin, conjugated to KLH. The corresponding sequence differs by a single amino acid in mouse and rat.
Application
Anti-Strumpellin antibody produced in rabbit may be used in immunoblotting.
Biochem/physiol Actions
Strumpellin is employed to the endosomes by interacting with the retromer complex for regulating actin. Mutations in the gene encoding strumpellin, KIAA0196 leads to hereditary spastic paraplegia, a progressive neurodegenerative disorder. 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.
Storage and Stability
For continuous use, store at 2-8 °C for up to one month. For extended storage freeze in working aliquots. Repeated freezing and thawing 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 12hours.
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.
The hereditary spastic paraplegia protein strumpellin: characterisation in neurons and of the effect of disease mutations on WASH complex assembly and function
Freeman C, et al.
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1832(1), 160-173 (2013)
Identification of KIAA0196 as a novel susceptibility gene for myofibril structural disorganization in cardiac development
Bu H, et al.
International Journal of Cardiology, 133(10), 2920-2941 (2020)
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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