Strumpellin Antibody

Product Specifications

Background

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.

NCBI Gene ID

9897

Swiss Prot

Q12768

Accession Number

NP_055661

Host

Rabbit

Reactivity

Human, Mouse, Rat

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Neuroscience

Purification

Strumpellin Antibody is affinity chromatography purified via peptide column.

Positive Control

Cat. No. 1316 - Human Ovary Tissue Lysate

Concentration

1 mg/mL

Buffer

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

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

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.

Fragment

IgG

Specificity

Multiple isoforms of Strumpellin are known to exist.

Symbol

KIAA0196

NCBI Official Name

KIAA0196

NCBI Organism

Homo sapiens

Background Reference 01

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.

Background Reference 02

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