ATXN1 Primary Antibody

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Description

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.Tissue specificity: Widely expressed throughout the body.

Product Overview
Entrez GenelD
6310
Aliases
ATX1; SCA1; D6S504E; ATXN1
Clone#
2F5
Host / Isotype
Mouse / IgG1
Species Reactivity
Human
Immunogen
Purified recombinant fragment of human ATXN1 expressed in E. Coli.
Formulation
Ascitic fluid containing 0.03% sodium azide.
Storage
4°C; -20°C for long term storage
Product Applications
WB (Western Blot)
1/500 - 1/2000
IHC_P(Immunohistochemistry)
1/200 - 1/1000
ICC (Immunocytochemistry)
1/200 - 1/1000
FCM (Flow Cytometry)
1/200 - 1/400
ELISA
1/10000
References
1. Nature. 2008 Apr 10;452(7188):713-8.
2. Biochem Biophys Res Commun. 2008 Jun 27;371(2):256-60.
3. Indian J Med Res. 2007 Nov;126(5):465-70.
Product Image
Western Blot
Figure 1: Western blot analysis using ATXN1 mAb against HEK293 (1) and ATXN1(AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate.
Figure 1: Western blot analysis using ATXN1 mAb against HEK293 (1) and ATXN1(AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate.
Immunohistochemical analysis
Figure 2: Immunohistochemical analysis of paraffin-embedded ovarian cancer tissues (left) and lung cancer tissues (right) using ATXN1 mouse mAb with DAB staining.
Figure 2: Immunohistochemical analysis of paraffin-embedded ovarian cancer tissues (left) and lung cancer tissues (right) using ATXN1 mouse mAb with DAB staining.
Immunofluorescence analysis
Figure 3: Immunofluorescence analysis of NTERA-2 cells using ATXN1 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor-555 phalloidin.
Figure 3: Immunofluorescence analysis of NTERA-2 cells using ATXN1 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor-555 phalloidin.
Flow cytometric
Figure 4: Flow cytometric analysis of Jurkat cells using ATXN1 mouse mAb (green) and negative control (purple).
Figure 4: Flow cytometric analysis of Jurkat cells using ATXN1 mouse mAb (green) and negative control (purple).
For Research Use Only. Not for use in diagnostic procedures.