Thermo Fisher Scientific SPG11 Polyclonal Antibody

Applications

Immunohistochemistry (Paraffin) (IHC (P))

• Tested Dilution: 1:50–1:200

Product Specifications

Product Specific Information

Immunogen sequence:
RLHNMKLSIS YLRECAKAND WLQFIIHSQL HNYHPAEVKS LIQYFSPVIQ DHLRLAFENL PSVPTSKMDS DQVCNKCPQE LQGSKQEMTD LFEILLQCS

• Highest antigen sequence identity to orthologs:
  • Mouse: 65%
  • Rat: 66%

Target Information

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) characterized by mental retardation and muscle stiffness at onset, 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 phosphorylated upon DNA damage, expressed throughout the brain with high levels in the cerebellum.
SPG11 mutations may occur more frequently in familial than sporadic forms of cHSP without TCC. Kjellin syndrome is associated with mutations in 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.

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