Invitrogen™ Ataxin 1 Recombinant Rabbit Monoclonal Antibody (16H8L13)

Rabbit Recombinant Monoclonal Antibody

Brand:  Invitrogen™ 703273

Product Code. 17106288

Description

This antibody is predicted to react with Mouse, Bovine.

The autosomal dominant cerebellar ataxias (ADCA) are a heterogeneous group of neurodegenerative disorders characterized by progressive degeneration of the cerebellum, brain stem and spinal cord. Clinically, ADCA has been divided into three groups: ADCA types I-III. ADCAI is genetically heterogeneous, with five genetic loci, designated spinocerebellar ataxia (SCA) 1, 2, 3, 4 and 6, being assigned to five different chromosomes. ADCAII, which always presents with retinal degeneration (SCA7), and ADCAIII often referred to as the 'pure' cerebellar syndrome (SCA5), are most likely homogeneous disorders. Several SCA genes have been cloned and shown to contain CAG repeats in their coding regions. ADCA is caused by the expansion of the CAG repeats, producing an elongated polyglutamine tract in the corresponding protein. The expanded repeats are variable in size and unstable, usually increasing in size when transmitted to successive generations. The function of the ataxins is not known. This locus has been mapped to chromosome 6, and it has been determined that the diseased allele contains 41-81 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). At least two transcript variants encoding the same protein have been found for this gene.

Specifications

• Antigen: Ataxin 1
• Classification: Recombinant Monoclonal
• Concentration: 0.5 mg/mL
• Formulation: PBS with 0.09% sodium azide; pH 7.4
• Gene Accession No.: P54253, Q63540
• Gene Symbols: ATXN1
• Immunogen: Protein corresponding to human Ataxin 1 [aa562-aa693].
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human, Rat
• Product Type: Antibody
• Isotype: IgG

• Applications: Western Blot, Immunocytochemistry
• Clone: 16H8L13
• Conjugate: Unconjugated
• Gene: ATXN1
• Gene Alias: 2900016G23Rik; alternative ataxin1; ataxin 1; Ataxin1; ataxin-1; Atx1; Atx-1; Atx-1-PB; Atxn1; C85907; CG4547; CG4547-PB; D6S504E; dAtx-1; Dmel\CG4547; Dmel_CG4547; ENSMUSG00000074917; Gm10786; OTTHUMP00000016065; SCA1; spinocerebellar ataxia 1; spinocerebellar ataxia 1 homolog; spinocerebellar ataxia type 1 protein; Spinocerebellar ataxia type 1 protein homolog
• Host Species: Rabbit
• Purification Method: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 25049, 6310
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid