产品名称
STRUM Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/9897
Human Swissprot No.
Q12768
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q12768/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/223593
Mouse Swissprot No.
Q8C2E7
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q8C2E7
免疫原
Synthesized peptide derived from human STRUM AA range: 515-565
特异性
This antibody detects endogenous levels of STRUM at Human/Mouse
稀释度
WB 1:500-2000;IHC-p 1:50-300
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Expressed ubiquitously.
细胞定位
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. .
功能
disease:Defects in KIAA0196 are the cause of spastic paraplegia autosomal dominant type 8 (SPG8) [MIM:603563]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.,similarity:Belongs to the strumpellin family.,tissue specificity:Expressed ubiquitously.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
