SPG11 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Supplier:  Proteintech Group Inc 165551AP150UL

Catalog No. 50-173-1023

Description

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.

Specifications

• Antigen: SPG11
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: SPG11
• Gene Alias: KIAA1840, Spastic paraplegia 11 protein, SPATACSIN, SPG11
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 214585, 311372, 80208
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin)
• Concentration: 0.27 mg/mL
• Formulation: PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
• Gene Accession No.: Q3UHA3, Q96JI7
• Gene Symbols: Spg11
• Immunogen: SPG11 Fusion Protein Ag9769
• Quantity: 150 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG