Strumpelline-antilichaam

Strumpellin Antibody: 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.

9897

Q12768

NP_055661

Human, Mouse, Rat

Unconjugated

Primary Antibodies

Neuroscience

Strumpellin Antibody is affinity chromatography purified via peptide column.

Cat. No. 1316 - Human Ovary Tissue Lysate

1 mg/mL

Strumpellin Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

Strumpellin antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

IgG

Multiple isoforms of Strumpellin are known to exist.

KIAA0196

KIAA0196

Homo sapiens

Valmanis PN, Meijer IA, Reynolds A, et al. Mutations in the KIAA0196 gene at the SPG8 locus cause hereditary spastic paraplegia. Am. J. Hum. Gen. 2007; 80:152-61.

Clemen CS, Tangavelou K, Strucksberg KH, et al. Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases. Brain 2010; 133:2920-41

Strumpellin Antibody: RTSC, SPG8, WASH complex subunit strumpellin

ELISA, WB

120952851

Liquid