SPG11
Reactivity: Human
WB
Host: Mouse
Polyclonal
unconjugated
Restrictions
For Research Use only
Format
Liquid
Buffer
PBS containing 0.02 % sodium azide.
Preservative
Sodium azide
Precaution of Use
WARNING: Reagents contain sodium azide. Sodium azide is very toxic if ingested or inhaled. Avoid contact with skin, eyes, or clothing. Wear eye or face protection when handling. If skin or eye contact occurs, wash with copious amounts of water. If ingested or inhaled, contact a physician immediately. Sodium azide yields toxic hydrazoic acid under acidic conditions. Dilute azide-containing compounds in running water before discarding to avoid accumulation of potentially explosive deposits in lead or copper plumbing.
Handling Advice
Avoid freezing and thawing repeatly.
Storage
4 °C/-20 °C
Storage Comment
Store at 4 °C for short term use.Store at -20 °C for long term preservation.
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: Spastic paraplegia 11, colorectal carcinoma-associated protein, spatacsin