Western Blot (WB)
(Figure 1: Western blot analysis using ATXN1 mAb against HEK293 (1) and ATXN1(AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate.)
Mouse anti-Human ATXN1 Monoclonal Antibody | anti-ATXN1 antibody
ATXN1 Antibody
WB: 1:500-1:2000
IHC: 1:200-1:1000
ICC: 1:200-1:1000
FC/FACS: 1:200-1:400
Western Blot (WB)
(Figure 1: Western blot analysis using ATXN1 mAb against HEK293 (1) and ATXN1(AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate.)
Western Blot (WB)
(Figure 1: Western blot analysis using ATXN1 mAb against HEK293 (1) and ATXN1(AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate.)
Function: Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism (PubMed:21475249). In concert with CIC and ATXN1L, involved in brain development (By similarity).
Subunit Structure: Homooligomer (PubMed:9097953). Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L and USP7 (PubMed:9353121, PubMed:11001934, PubMed:12062018, PubMed:12093161, PubMed:16121196). Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding (PubMed:21475249). Found in a complex with CIC and ATXN1L (By similarity).
Post-translational Modifications: Ubiquitinated by UBE3A, leading to its degradation by the proteasome. The presence of expanded poly-Gln repeats in spinocerebellar ataxia 1 (SCA1) patients impairs ubiquitination and degradation, leading to accumulation of ATXN1 in neurons and subsequent toxicity. Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract. Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.
Similarity: The AXH domain is required for interaction with CIC. Belongs to the ATXN1 family.
NCBI and Uniprot Product Information
Predicted: 87 kDa
NCBI 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 40-83 CAG repeats, compared to 6-39 in the normal allele, and is associated with spinocerebellar ataxia type 1 (SCA1). Alternative splicing results in multiple transcript variants, with one variant encoding multiple distinct proteins, ATXN1 and Alt-ATXN1, due to the use of overlapping alternate reading frames. [provided by RefSeq, Nov 2017]
Uniprot Description
Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism (PubMed:21475249). In concert with CIC and ATXN1L, involved in brain development ().
Research Articles on ATXN1
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Product Notes
The ATXN1 atxn1 (Catalog #AAA9602500) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The ATXN1 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ATXN1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemisty (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC), ELISA (EIA), Flow Cytometry (FC/FACS). ELISA: 1:10000 WB: 1:500-1:2000 IHC: 1:200-1:1000 ICC: 1:200-1:1000 FC/FACS: 1:200-1:400. Researchers should empirically determine the suitability of the ATXN1 atxn1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ATXN1, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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