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Product Details
MA5-48595
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse
Host/Isotype
Mouse
/ IgG1
Class
Monoclonal
Type
Antibody
Clone
2B8A2
Immunogen
Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli.
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein G
Storage buffer
PBS
Contains
0.05% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Wet ice
RRID
AB_3093326
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: alternative ataxin1; Ataxin-1; OTTHUMP00000016065; spinocerebellar ataxia 1 homolog; Spinocerebellar ataxia type 1 protein; Spinocerebellar ataxia type 1 protein homolog
Gene Aliases: 2900016G23Rik; ATX1; ATXN1; C85907; D6S504E; ENSMUSG00000074917; Gm10786; SCA1
UniProt ID: (Human) P54253, (Mouse) P54254
Entrez Gene ID: (Human) 6310, (Mouse) 20238
negative regulation of transcription from RNA polymerase II promoter
transcription, DNA-templated
regulation of transcription, DNA-templated
adult locomotory behavior
visual learning
negative regulation of phosphorylation
negative regulation of insulin-like growth factor receptor signaling pathway
negative regulation of transcription, DNA-templated
positive regulation of transcription from RNA polymerase II promoter
lung alveolus development
nuclear export
excitatory postsynaptic potential
positive regulation of glial cell proliferation
RNA processing
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