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生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
形狀
buffered aqueous solution
物種活性
mouse, human, rat
技術
immunohistochemistry: suitable
indirect ELISA: suitable
western blot: suitable
NCBI登錄號
UniProt登錄號
運輸包裝
dry ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... SPG11(80208)
免疫原
SPG11 antibody was raised against a 15 amino acid peptide of human SPG11.
特點和優勢
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
標靶描述
Hereditary spastic paraplegias (HSPs) are genetically and phenotypically heterogeneous disorders. Spastic paraplegia with thinning of the corpus callosum (ARHSP-TCC) is a relatively frequent form of complicated hereditary spastic paraplegia (cHSP) in which mental retardation and muscle stiffness at onset are followed by slowly progressive paraparesis and cognitive deterioration. Mutations of the SPG11 gene encoding the spatacsin protein have been identified as a major cause of HSP-TCC. Spatacsin is a potential transmembrane protein that is phosphorylated upon DNA damage. It is expressed in all structures of the brain, with a high expression in the cerebellum. SPG11 mutations may occur more frequently in familial than sporadic forms of cHSP without TCC. Kjellin syndrome is found to be associated with mutations in not only the SPG15 gene but also SPG11 gene. Recent studies show Parkinsonism may initiate SPG11-linked HSP TCC and that SPG11 may cause juvenile Parkinsonism.
聯結
The action of this antibody can be blocked using blocking peptide SBP3500003.
外觀
Supplied at approx. 1 mg/mL in phosphate buffered saline containing 0.02% sodium azide.
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相關產品
儲存類別代碼
10 - Combustible liquids
水污染物質分類(WGK)
WGK 2
閃點(°F)
Not applicable
閃點(°C)
Not applicable
Proceedings of the National Academy of Sciences of the United States of America, 120(51), e2300681120-e2300681120 (2023-12-15)
Idiopathic normal pressure hydrocephalus (iNPH) is an enigmatic neurological disorder that develops after age 60 and is characterized by gait difficulty, dementia, and incontinence. Recently, we reported that heterozygous CWH43 deletions may cause iNPH. Here, we identify mutations affecting nine
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