Invitrogen

Human SPG11 Synthetic Peptide

产品信息

PEP-0799

应用

建议稀释比

已发表文章

对照试剂 (Ctrl)

Assay-dependent

阻断反应 (BLOCK)

Assay-dependent

产品规格

种属反应

Human

分类

Synthetic

类型

Peptide

偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

200 µg/mL

纯化类型

purified

保存液

PBS, pH 7.2, with 0.1% BSA

内含物

0.02% sodium azide

保存条件

-20°C

产品详细信息

This peptide corresponds to 15 amino acids near the carboxy terminus of human SPG11.

PEP-0799 can be used as a blocking peptide with polyclonal antibody PA5-20683.

靶标信息

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.

仅用于科研。不用于诊断过程。未经明确授权不得转售。