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Merck

SAB3500003

Sigma-Aldrich

Anti-SPG11 antibody produced in rabbit

affinity isolated antibody, buffered aqueous solution

别名:

Anti-Colorectal carcinoma-associated protein, Anti-Spastic paraplegia 11, Anti-Spatacsin

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.41

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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说明
价格

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


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Hong Wei Yang et al.
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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