Rabbit Polyclonal antibody to Ataxin 1 (ataxin 1)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. At least two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq]
Immunogen
Recombinant protein encompassing a sequence within the center region of human Ataxin 1. The exact sequence is proprietary.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WB
*Optimal dilutions/concentrations should be determined by the researcher.
:
Species Reactivity
Mouse
Predicted Reactivity
Human, RatHuman (100%), Rat (81%)
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Gene Synonyms
ataxin 1 ATXN1 ATX1 D6S504E SCA1
Target
Ataxin 1
Molecular Weight(MW)
87 kDa (note) (2)
Cellular Localization
Cytoplasm , Nucleus
Application
Application
Mouse tissue extract (50 μg) was separated by 7.5% SDS-PAGE, and the membrane was blotted with Ataxin 1 antibody diluted at 1:500.
Positive Control
mouse brain
Application Notes
*Optimal dilutions/concentrations should be determined by the researcher.
:
Additional Information
Form
Liquid
Storage Instructions
Keep as concentrated solution. Aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
Storage Buffer
1XPBS, 1% BSA, 20% Glycerol (pH7). 0.025% ProClin 300 was added as a preservative.