STRUM rabbit pAb

This gene encodes a 134 kDa protein named strumpellin that is predicted to have multiple transmembrane domains and a spectrin-repeat-containing domain. This ubiquitously expressed gene has its highest expression in skeletal muscle. The protein is named for Strumpell disease; a form of hereditary spastic paraplegia (HSP). Spastic paraplegias are a diverse group of disorders in which the autosomal dominant forms are characterized by progressive, lower extremity spasticity caused by axonal degeneration in the terminal portions of the longest descending and ascending corticospinal tracts. More than 30 loci (SPG1-33) have been implicated in hereditary spastic paraplegia diseases. [provided by RefSeq, Aug 2009],

Product Specifications

Background

This gene encodes a 134 kDa protein named strumpellin that is predicted to have multiple transmembrane domains and a spectrin-repeat-containing domain. This ubiquitously expressed gene has its highest expression in skeletal muscle. The protein is named for Strumpell disease; a form of hereditary spastic paraplegia (HSP) . Spastic paraplegias are a diverse group of disorders in which the autosomal dominant forms are characterized by progressive, lower extremity spasticity caused by axonal degeneration in the terminal portions of the longest descending and ascending corticospinal tracts. More than 30 loci (SPG1-33) have been implicated in hereditary spastic paraplegia diseases. [provided by RefSeq, Aug 2009]

UniProt

Q12768

Swiss Prot

Q12768

Reactivity

Human; Mouse

Immunogen

Synthesized peptide derived from human STRUM AA range: 515-565

Target

STRUM

Clonality

Polyclonal

Source

Rabbit

Applications

WB; IHC

Concentration

1 mg/ml

Dilution

WB 1:500-2000; IHC-p 1:50-300

Buffer

-20°C/1 year

Storage Conditions

-20°C/1 year

Fragment

IgG

Subcellular Location

Cytoplasm, cytosol . Endoplasmic reticulum . Early endosome . Colocalizes with SYP/synaptophysin in the external molecular layer of the dentate gyrus and in motoneurons of the ventral horn of spinal cord. .