600-401-ET2
SPG11 Antibody
Cannot supply to this region.
- SKU:
- 600-401-ET2
- Additional Names:
- SPG11|SPG11 Antibody, KIAA1840, KIAA1840, Spatacsin, Colorectal carcinoma-associated protein
- Application:
- ELISA, IHC, WB
- Concentration:
- 1 mg/ml
- Physical State:
- Liquid
- Species Reactivity:
- Human
- Storage Conditions:
- -20[o]C aliquoted. Aliquot. Avoid freeze/thaw cycles., 2-8[o]C diluted. Aliquot. Avoid freeze/thaw cycles.
- Supplier:
- Rockland Inc
- Host:
- Rabbit
- Reactivities:
- Human, Mouse, Rat
- Buffer:
- 0.01 M Sodium Phosphate, 0.25 M Sodium Chloride
- Immunogen:
- Anti-SPG11 antibody was prepared from whole rabbit serum produced by repeated immunizations with a 15 amino acid synthetic peptide near the C-terminus of human SPG11.
- Formulation:
- 0.01 M Sodium Phosphate, 0.25 M Sodium Chloride, pH 7.2
- Uniprot:
- Q96JI7
- Synonyms:
- ALS5;amyotrophic lateral sclerosis 5;CMT2X;colorectal carcinoma-associated protein;KIAA1840;spastic paraplegia 11 (autosomal recessive);spastic paraplegia 11 protein;spatacsin;SPG11, spatacsin vesicle trafficking associated
- Extra Details:
- 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.
- Shipping Conditions:
- Dry Ice