Thermo Fisher Scientific SPG11 Polyclonal Antibody

Applications and Tested Dilution

Product Specifications

Product Specific Information

• Suggested positive control: mouse heart tissue lysate
• PA5-20683 can be used with blocking peptide PEP-0799.

Target Information

Hereditary spastic paraplegias (HSPs) are genetically and phenotypically heterogeneous disorders.
Spastic paraplegia with thinning of the corpus callosum (ARHSP-TCC) is a frequent form of complicated hereditary spastic paraplegia (cHSP), characterized by mental retardation and muscle stiffness at onset, followed by slowly progressive paraparesis and cognitive deterioration.
Mutations in the SPG11 gene encoding the spatacsin protein are a major cause of HSP-TCC. Spatacsin is a potential transmembrane protein phosphorylated upon DNA damage and is highly expressed in the cerebellum.
SPG11 mutations are more frequent in familial than sporadic forms of cHSP without TCC. Kjellin syndrome is associated with mutations in both SPG15 and SPG11 genes.
Recent studies indicate that Parkinsonism may initiate SPG11-linked HSP TCC and that SPG11 may cause juvenile Parkinsonism.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.