146385
SPG11 (Spatacsin, Colorectal Carcinoma-associated Protein, Spastic Paraplegia 11 Protein, KIAA1840, FLJ21439, DKFZp762B1512, FLJ21439)
Size
£655.00
- SKU:
- 146385
- Purity:
- Purified by immunoaffinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Synthetic peptide corresponding to 15aa from near the C-terminus of human SPG11.
- Format:
- Supplied as a liquid in PBS, 0.02% sodium azide.
- 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. Applications: Suitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 0.5-1ug/ml Immunohistochemistry (Formalin fixed paraffin embedded): 2.5ug/ml Optimal dilutions to be determined by the researcher. Positive Control: Mouse Heart Tissue Lysate Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice