SPG11 Rabbit Polyclonal Antibody
Frequently bought together (1)
beta Actin Mouse Monoclonal Antibody, Clone OTI1, Loading Control
USD 200.00
Other products for "SPG11"
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) |
Database Link | |
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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