Full product name
Ataxin-1 Antibody
Code
BT-AP00685
Host
Rabbit
Isotype
IgG
Size
20ul,50ul,100ul
Clonality
Polyclonal
Immunogen
The antiserum was produced against synthesized peptide derived from human Ataxin 1. AA range:742-791
Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
1 mg/ml
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Storage
-20°C for one year
Background
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 40-83 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 ATXN1.
Uniprot accession
Molecular weight
87051
Gene ID
Human: 6310; Mouse: 20238
Synonyms
ATXN1; ATX1; SCA1; Ataxin-1; Spinocerebellar ataxia type 1 protein
Research area
Epigenetics and Nuclear Signaling
Target protein
ATXN1
