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SPG11 Antibody

     
  • WB - SPG11 Antibody ASC10847
    Western blot analysis of SPG11 in mouse heart tissue lysate with SPG11 antibody at (A) 0.5 and (B) 1 µg/mL.
    detail
  • IHC - SPG11 Antibody ASC10847
    Immunohistochemistry of SPG11 in mouse brain tissue with SPG11 antibody at 2.5 µg/mL.
    detail
  • SPECIFICATION
  • CITATIONS
  • PROTOCOLS
  • BACKGROUND
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, IHC-P, E
Primary Accession Q96JI7
Other Accession AAI53880, 158253417
Reactivity Human, Mouse, Rat
Host Rabbit
Clonality Polyclonal
Isotype IgG
Calculated MW 278868 Da
Application Notes SPG11 antibody can be used for detection of SPG11 by Western blot at 0.5 - 1 µg/mL. Antibody can also be used for immunohistochemistry starting at 2.5 µg/mL.
Additional Information
Gene ID 80208
Target/Specificity SPG11;
Reconstitution & Storage SPG11 antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
PrecautionsSPG11 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name SPG11
Synonyms KIAA1840
Function May play a role in neurite plasticity by maintaining cytoskeleton stability and regulating synaptic vesicle transport.
Cellular Location Cytoplasm, cytosol. Nucleus. Cell projection, axon. Cell projection, dendrite. Note=Mainly cytoplasmic
Tissue Location Expressed in all structures of brain, with a high expression in cerebellum. Expressed in cortical projection neurons
Citations (0)
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Background

SPG11 Antibody: 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.

References

Stevanin G, Azzedine H, Denora P, et al. Mutations in SPG11, encoding spatacsin, are a major cause of spastic paraplegia with thin corpus callosum. Nature Gen.2007; 39:366-372.
Paisan-Ruiz C, Dogu O, Yilmaz A, et al. SPG11 mutations are common in familial cases of complicated hereditary spastic paraplegia. Neurology2008; 70:1384-9. 
Crimella C, Arnoldi A, Crippa F, et al. Point mutations and a large intragenic deletion in SPG11 in complicated spastic paraplegia without thin corpus callosum. J. Med. Genet.2009; 46:345-51. 
Orlén H, Melberg A, Raininko R, et al. SPG11 mutations cause Kjellin syndrome, a hereditary spastic paraplegia with thin corpus callosum and central retinal degeneration. Am. J. Med. Genet. B Neuropsychiatr. Genet.2009; epub. 

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$ 379.00
Cat# ASC10847
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