BackgroundThe 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.
Product Information
Clone Number2F5
SpeciesMouse IgG1
Entrez Gene6310
Recommended DilutionsWB: 1/500 - 1/2000, IHC: 1/200 - 1/1000, IF: 1/200 - 1/1000, FC: 1/200-1/400
ELISA: Propose dilution 1/10000.
Molecular Weight87kDa
All Names
AlternameATX1; SCA1; D6S504E; ATXN1
ImmunogenPurified recombinant fragment of human ATXN1 expressed in E. Coli.
Storage/StabilityStore at 4℃, for long term storage, store at -20℃.
Form Or Antibody
Form of AntibodyAscitic fluid containing 0.03% sodium azide.
Research AreaAutophagy antibody Cancer Cardiovascular Cell Biology Epigenetics & Nuclear Signaling Developmental Biologys Immunology Drug Discovery Products Metabolism Neuroscience Signal Transduction Stem Cells