ATXN7 Antibody
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Description
Introduction to ATXN7 Antibody
ATXN7 Antibody is a laboratory reagent used to detect ataxin-7, a core component of the SAGA chromatin remodeling complex. Its primary applications include immunoprecipitation, immunofluorescence microscopy, and Western blotting to study protein localization, interactions, and post-translational modifications (PTMs). These antibodies target epitopes in the full-length protein or specific domains, such as the N-terminal polyglutamine (polyQ) tract or regions involved in microtubule binding .
Applications in Research
ATXN7 Antibody is employed in multiple experimental contexts to elucidate its physiological and pathological roles:
Immunoprecipitation
Used to isolate ATXN7 and its interacting partners:
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Key Findings:
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ATXN7 interacts with α-tubulin via residues 120–230, stabilizing microtubules .
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PolyQ-expanded ATXN7 retains interaction with SAGA complex subunits (e.g., Trrap, Gcn5) but disrupts histone deubiquitination .
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Caspase 7 cleavage sites in ATXN7 release the deubiquitination module (DUBm) from SAGA, impairing transcriptional regulation .
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Immunofluorescence Microscopy
Visualizes subcellular localization and aggregation:
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Key Findings:
Western Blotting
Assesses protein stability and PTMs:
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Key Findings:
Interaction with Microtubules
ATXN7 binds α-tubulin, stabilizing cytoskeletal structures. PolyQ expansion does not disrupt this interaction but reduces cytoplasmic ATXN7 levels, leading to microtubule instability .
Role in SCA7 Pathology
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Nuclear Inclusions: PolyQ ATXN7 aggregates in nuclei, impairing transcriptional regulation and causing photoreceptor degeneration .
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Chromatin Dysregulation: Mutant ATXN7 alters histone modifications (e.g., H2B monoubiquitination) and disrupts recruitment of SAGA complexes to target genes .
Impact on Chromatin Remodeling
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TFTC/STAGA Complex: PolyQ ATXN7 does not reduce histone acetyltransferase (HAT) activity but disrupts chromatin organization, leading to misregulated gene expression .
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Mitochondrial Dysfunction: ATXN7 acetylation and aggregation correlate with impaired oxidative phosphorylation and mitochondrial fragmentation .
Table 1: ATXN7 Antibody Applications and Findings
Table 2: Functional Impacts of ATXN7 Antibody Studies
Product Specs
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Typically, we can ship the products within 1-3 business days after receiving your orders. Delivery times may vary depending on the purchasing method or location. Please consult your local distributors for specific delivery timeframes.
Synonyms
ATXN7 antibody; SCA7Ataxin-7 antibody; Spinocerebellar ataxia type 7 protein antibody
Target Names
ATXN7
Uniprot No.
Target Background
Function
Ataxin-7 (ATXN7) is a component of the STAGA transcription coactivator-HAT complex. It mediates the interaction of the STAGA complex with the CRX protein and plays a role in CRX-dependent gene activation. Additionally, ATXN7 is essential for microtubule cytoskeleton stabilization.
Gene References Into Functions
- Genetic testing revealed the presence of 48 CAG repeats within one ATXN7 gene in a case of spinocerebellar ataxia type 7 (SCA7). PMID: 28645341
- Our observations indicated that individuals carrying either ATXN7 or TBP alleles with relatively large CAG repeat sizes in both alleles had a significantly increased risk of lifetime depression. PMID: 28585930
- The intronic SNP rs6798742 has been associated with ATXN7 CAG-region expansion. PMID: 28597910
- ATXN7 might be a potential predictor of post-operative prognosis in patients with Hepatitis B Virus-related hepatocellular carcinoma. PMID: 27855399
- A South American cohort study did not confirm the effect of four candidate loci as modifiers of onset age: mitochondrial A10398G polymorphism and CAGn at RAI1, CACNA1A, ATXN3, and ATXN7 genes. PMID: 25869926
- Our research provided a clinico-genetic analysis of nine Indian SCA7 families. Additionally, CAG repeat distribution analysis in diverse Indian populations revealed the occurrence of ATXN7-CAG intermediate alleles in a predisposed population. PMID: 25900954
- Data suggest that aggregates formed by polyQ-expanded ataxin 7 sequester ubiquitin-specific protease (USP22) through specific interactions. PMID: 26195632
- The accumulation of insoluble aggregates of two pathological polyglutamine proteins, truncated Ataxin-7 and full-length Ataxin-3, beyond a critical threshold may be responsible for neurotoxicity. PMID: 26210447
- The proband exhibited a typical phenotype of SCA7, including cone dystrophy and spinocerebellar ataxia. PMID: 25643591
- Research suggests that sequestration of both enzymatic centers in SAGA upon ATXN7 poly(Q) expansion likely contributes to the development and progression of spinocerebellar ataxia type 7. PMID: 25755283
- This study demonstrated evidence in vivo, using the SCA7 KI mouse model, that progressive accumulation of mutant ataxin-7 impairs autophagy. PMID: 24859968
- Epidemiological evidence suggests a SCA7 founder effect in a Mexican population with spinocerebellar ataxia. PMID: 23368522
- Analysis of the founder effect and ancestral origin of the spinocerebellar ataxia type 7 mutation in Mexican families. PMID: 24374739
- Haplotype and phylogenetic analyses provide evidence suggesting that the relatively high frequency of SCA7 in the Mexican population is the result of a founder mutation. Additionally, Mexican SCA7 carriers exhibit Western European ancestry. PMID: 23828024
- PolyQ-expanded ataxin-7 directly bound the Gcn5 catalytic core of SAGA while in association with its regulatory proteins, Ada2 and Ada3. PMID: 24129567
- The results demonstrated that a common genetic variant in the ataxia-causing gene ATXN7 influences cerebellar grey matter volume in healthy young adults. PMID: 23100044
- Sequestration of the ponsin splice variant R85FL by the polyglutamine-expanded Atx7 in cells is mediated by the specific SH3C-PRR interaction, which is implicated in the pathogenesis of spinocerebellar ataxia 7. PMID: 23892081
- The role of ataxin-7 in the differentiation of photoreceptors and cerebellar neurons. PMID: 23226359
- Full-length and cleaved fragments of the SCA7 disease protein ataxin-7 (ATXN7) are differentially degraded in a spinocerebellar ataxia type 7 rat model. PMID: 22367614
- Critical nuclear events lead to transcriptional alterations in polyglutamine diseases such as spinocerebellar ataxia type 7 (SCA7) and Huntington's disease (HD). PMID: 22917585
- The results of this study demonstrated that oxidative stress contributes to ATXN7 aggregation as well as toxicity. PMID: 22827889
- ATXN7 distribution frequently shifts from the nucleus to the cytoplasm; cytoplasmic ATXN7 associates with microtubules (MTs); expression of ATXN7 stabilizes MTs; findings provide a novel physiological function of ATXN7 in regulation of cytoskeletal dynamics and suggest that abnormal cytoskeletal regulation may contribute to SCA7 disease pathology. PMID: 22100762
- The results of this study indicated that SCA7 disease pathogenesis involves a convergence of alterations in a variety of different cell types to fully recapitulate the cerebellar degeneration. PMID: 22072678
- The Trinucleotide Repeat Expansion mutation in ATXN7 is related to Spinocerebellar ataxia type 7. PMID: 21827908
- The interaction between APLP2 and ataxin-7 and proteolytic processing of APLP2 may contribute to the pathogenesis of spinocerebellar ataxia type 7. PMID: 20732423
- Researchers identified 118 protein interactions for CACNA1A and ATXN7, linking them to other ataxia-causing proteins and the ataxia network. Notably, the ataxia network is significantly enriched for proteins that interact with known macular degeneration-causing proteins. PMID: 21078624
- In response to polyglutamine toxicity, transgenic murine SCA7 rods undergo a range of radically different cell fates correlating with the nature, level, and ratio of mutant transgene ATXN7 species. PMID: 20600911
- The solution structures of the SCA7 domain of both ATXN7 and ATXN7L3 reveal a new, common zinc-finger motif at the heart of two distinct folds, providing a molecular basis for the observed functional differences. PMID: 20634802
- This study suggested that the SCA7 gene alteration in SCA7 patients in a Chinese Han family. PMID: 20739808
- These results demonstrate an influence of SUMOylation on the multistep aggregation process of ATXN7 and implicate a role for ATXN7 SUMOylation in SCA7 pathogenesis. PMID: 19843541
- Analysis of RNA hairpins selective for silencing the mutant ataxin-7 transcript. PMID: 19789634
- CAG expansion in the SCA7 locus is associated with Machado-Joseph disease. PMID: 11697524
- Activated caspase-3 was recruited into the inclusions in both the cell models and human SCA7 brain, and its expression was upregulated in cortical neurons. PMID: 11709544
- Expression of ataxin-7 in CNS and non-CNS tissue of normal and SCA7 individuals. PMID: 12070661
- Identification of a novel ataxin-7 protein enriched in the central nervous system suggests that expression of multiple polyglutamine-containing proteins may play a role in the neurodegeneration patterns characteristic of SCA7. PMID: 12533095
- This study demonstrates that ataxin-7 is the human orthologue of the yeast SAGA SGF73 subunit and is a bona fide subunit of the human TFTC-like transcriptional complexes. PMID: 15115762
- This patient presented with Spinocerebellar Ataxia 7 due to unique instability of the CAG repeat. PMID: 15316811
- Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity, leading to retinal degeneration in Spinocerebellar ataxia type 7. PMID: 15932940
- Research shows that transcription mediated by both CBP and RORalpha1 was repressed by expanded ataxin-7. Ataxin-7 may act as a repressor of transcription by inhibiting the acetylation activity of TFTC and STAGA. PMID: 15936949
- Ataxin-90A aggregates differed morphologically from ataxin7 - 100Q aggregates and were more toxic to mesencephalic neurons, suggesting that toxicity was determined by the type of aggregate rather than the cellular misfolding response. PMID: 16325416
- Trinucleotide repeat expansions of ataxin 7 may be involved in neurodegenerative diseases such as cerebellar ataxia. PMID: 16962040
- Origin of the SCA7 gene mutation in South Africa and the possibility of a founder effect in the Black population. PMID: 17026624
- Massive SCA7 expansion detected in a 7-month-old male with hypotonia, cardiomegaly, and renal failure. PMID: 17254003
- We present a pediatric patient with 13 and 70 trinucleotide CAG repeats within the SCA7 gene and no family history, whose presentation mimicked Kearns-Sayre syndrome (KSS). PMID: 17720198
- Expanded CAG-repeats in the SCA7 gene were observed within members of a large Chinese family with spinocerebellar ataxia. PMID: 18325672
- Patients with genetically confirmed SCA 7 presented with early macular dysfunction, preceding any signs of abnormalities in fundus appearance. PMID: 19172503
- Ataxin-7 is the human orthologue of SGF73, which is a subunit of the yeast SAGA complex, a coactivator required for transcription of a subset of RNA Pol II-dependent genes. Ataxin-7 is a new subunit of the mammalian SAGA-like complexes, TFTC/STAGA. PMID: 15115762
Database Links
Involvement In Disease
Spinocerebellar ataxia 7 (SCA7)
Protein Families
Ataxin-7 family
Subcellular Location
[Isoform a]: Nucleus. Nucleus, nucleolus. Nucleus matrix. Cytoplasm, cytoskeleton. Note=In addition to a diffuse distribution throughout the nucleus, it is associated with the nuclear matrix and the nucleolus. It is able to shuttle between the nucleus and cytoplasm.; [Isoform b]: Cytoplasm.
Tissue Specificity
Isoform a and isoform b are expressed in CNS, but isoform a is expressed predominantly in the peripherical tissues. Isoform b is also highly expressed in the frontal lobe, skeletal muscle and spinal cord and is expressed at a lower level in the lung, lymp
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