Ataxin 1 Monoclonal Antibody (S65-37), PE, Invitrogen™

Description

• Rat: 93% identity (15/16 amino acids identical)
• Human: 87% identity (14/16 amino acids identical)
• 1 μg/mL of MA5-45708 was sufficient for detection of Ataxin 1 in 20 μg of COS cells transiently transfected with Ataxin-1 lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody.∣Detects approximately 85kDa
• No cross-reactivity against phosphor S751-Ataxin-1
• Minimal cross-reactivity against S751A mutant of Ataxin-1 by ELISA and immunofluorescence and negative by immunoblot
• 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.