SPG11 Rabbit Polyclonal Antibody

CAT#: TA306723

Rabbit Polyclonal SPG11 Antibody


USD 570.00

5 Days*

Size
    • 100 ug

Product Images

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Specifications

Product Data
Applications IHC, WB
Recommended Dilution WB: 0.5 - 1 ug/mL, ICC: 2.5 ug/mL
Reactivities Human, Mouse, Rat
Host Rabbit
Isotype IgG
Clonality Polyclonal
Immunogen SPG11 antibody was raised against a 15 amino acid peptide of human SPG11.
Formulation PBS containing 0.02% sodium azide.
Concentration 1ug/ul
Purification Affinity chromatography purified via peptide column
Conjugation Unconjugated
Storage Store at -20°C as received.
Stability Stable for 12 months from date of receipt.
Gene Name spastic paraplegia 11 (autosomal recessive)
Background 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.
Synonyms KIAA1840
Reference Data

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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.