Strumpellin is a ubiquitously expressed, multi-transmembrane and spectrin-repeat-containing protein. It is named for Strumpell disease; a progressive upper-motor neurodegenerative disease termed hereditary spastic paraplegia (HSP). The Strumpellin gene maps to the eighth HSP locus (SPG8) on chromosome 8p24.13. Three families liked to this locus have mutations in the Strumpellin gene; rescue studies of zebrafish with decreased Strumpellin expression with Strumpellin mRNA containing these mutations showed impaired normal function of this protein. Recent studies suggest that Strumpellin may also be involved in protein aggregation diseases.
应用类型
ELISA,Western Blot,
免疫原
Anti-Strumpellin antibody was prepared from whole rabbit serum produced by repeated immunizations with a 19 amino acid synthetic peptide near the C-terminus of human Strumpellin.
来源宿主
Rabbit
反应性
H. sapiens (Human); Mus musculus (Mouse); Rattus (Rat)
保存建议
Store vial at -20° C prior to opening. Aliquot contents and freeze at -20° C or below for extended storage. Avoid cycles of freezing and thawing. Centrifuge product if not completely clear after standing at room temperature. This product is stable for several weeks at 4° C as an undiluted liquid. Dilute only prior to immediate use.
