Invitrogen™ SPG11 Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA5115957

Product Code. 17298733

Description

Antibody detects endogenous levels of total SPG11.

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: 6030465E24Rik; A330015I11; ALS5; C530005A01Rik; CMT2X; Colorectal carcinoma-associated protein; KIAA1840; RGD1562529; spastic paraplegia 11; spastic paraplegia 11 (autosomal recessive); spastic paraplegia 11 protein; Spastic paraplegia 11 protein homolog; spatacsin; Spg11
• Host Species: Rabbit
• Purification Method: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 214585, 311372, 80208
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide
• Gene Accession No.: Q3UHA3, Q96JI7
• Gene Symbols: SPG11
• Immunogen: A synthesized peptide derived from human SPG11(Accession Q96JI7), corresponding to amino acid residues I507-N557.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG