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

Rabbit Polyclonal Antibody

Product Code	Brand	Quantity
16836214	Proteintech 16555-1-AP-20UL	20 μL
16826214	Proteintech 16555-1-AP-150UL	150 μL

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
• Applications: Immunohistochemistry (Paraffin)
• Conjugate: Unconjugated
• Host Species: Rabbit
• Target Species: Human, Rat, Mouse
• Gene Accession No.: Q3UHA3, Q96JI7
• Gene ID (Entrez): 214585, 311372, 80208
• Immunogen: SPG11 Fusion Protein Ag9769
• Primary or Secondary: Primary
• Content And Storage: -20°C
• Gene: SPG11

• Concentration: 0.27 mg/mL
• Classification: Polyclonal
• Form: Liquid
• Regulatory Status: RUO
• Formulation: PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
• Gene Alias: KIAA1840, Spastic paraplegia 11 protein, SPATACSIN, SPG11
• Gene Symbols: Spg11
• Isotype: IgG
• Purification Method: Antigen Affinity Chromatography
• Product Type: Antibody