Mouse anti-Mouse, Rat ATXN7 Monoclonal Antibody | anti-ATXN7 antibody

ATXN7 (Ataxin-7)

Cat. Num. AAA633152

• Gene Names: ATXN7; SCA7; OPCA3; ADCAII; FLJ17787
• Reactivity: Mouse, Rat
• Applications: Western Blot
• Purity: Ascites; Ascites
• Synonyms: ATXN7; Monoclonal Antibody; ATXN7 (Ataxin-7); Anti -ATXN7 (Ataxin-7); anti-ATXN7 antibody

• Host: Mouse
• Reactivity: Mouse, Rat
• Clonality: Monoclonal
• Isotype: IgG1k
• Clone Number: 10H27
• Specificity: Recognizes human Ataxin-7 protein. Species Crossreactivity: rat, mouse.
• Purity/Purification: Ascites; Ascites
• Form/Format: Supplied as a liquid, no preservatives.

• Applicable Applications for anti-ATXN7 antibody: Western Blot (WB)
• Application Notes: Suitable for use in Western Blot.; Dilution: Western Blot: 1:1000
• Immunogen: Recombinant human Ataxin 7 as a fusion protein (aa1- 229).
• Positive Control: Rat brain tissue lysate.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blotting analysis:|Representative Lot Data.|Rat brain lysate was resolved by electrophoresis, transferred to a PVDF membrane and probed with a 1:1,000 dilution of Anti-Ataxin-7, clone 3SCA-1C1 antibody. |Proteins were visualized using a HRP conjugated Goat anti-Mouse secondary antibody and visualized using a chemiluminescence detection system. |Arrow indicates Ataxin-7 (~96 kDa).)

Related Product Information for anti-ATXN7 antibody

Ataxin-7 is a protein of unknown function. However, since this protein contains a nuclear localization sequence and is found to be localized in the nucleus, it has been postulated to be a potential transcription factor. Spinocerebellar ataxia-7 (one of a group of hereditary neurodegenrative diseases) is caused by an expanded CAG repeat in the gene encoding ataxin-7 (the diseased allele associated with Spinocerebellar ataxia-7, contains 38-130 CAG repeats (near the N-terminus), compared to 7-17 in the normal allele). In cells where there is a mutation of the SCA7 gene, ataxin-7 accumulates in intranuclear inclusions and can result in cell death. Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs.

Product Categories/Family for anti-ATXN7 antibody

Antibodies; Abs to Ataxin

NCBI and Uniprot Product Information

• NCBI GI #: 293651613
• NCBI GeneID: 6314
• NCBI Accession #: NP_001170858.1
• NCBI GenBank Nucleotide #: NM_001177387.1
• UniProt Accession #: O15265; O75328; O75329; Q9Y6P8; O43180; Q8TEZ8; Q9UPD8
• Molecular Weight: 95,451 Da
• NCBI Official Full Name: ataxin-7 isoform b
• NCBI Official Synonym Full Names: ataxin 7
• NCBI Official Symbol: ATXN7
• NCBI Official Synonym Symbols: SCA7; OPCA3; ADCAII; FLJ17787
• NCBI Protein Information: ataxin-7; OTTHUMP00000213403; OTTHUMP00000213405; OTTHUMP00000227997; spinocerebellar ataxia type 7 protein
• UniProt Protein Name: Ataxin-7
• Protein Family: Ataxin
• UniProt Gene Name: ATXN7
• UniProt Synonym Gene Names: SCA7
• UniProt Entry Name: ATX7_HUMAN

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. This locus has been mapped to chromosome 3, and it has been determined that the diseased allele associated with spinocerebellar ataxia-7 contains 38-130 CAG repeats (near the N-terminus), compared to 7-17 in the normal allele. The encoded protein is a component of the SPT3/TAF9/GCN5 acetyltransferase (STAGA) and TBP-free TAF-containing (TFTC) chromatin remodeling complexes, and it thus plays a role in transcriptional regulation. Alternative splicing results in multiple transcript variants. [provided by RefSeq]

Uniprot Description

ataxin-7: Acts as component of the STAGA transcription coactivator-HAT complex. Mediates the interaction of STAGA complex with the CRX and is involved in CRX-dependent gene activation. Necessary for microtubule cytoskeleton stabilization. Defects in ATXN7 are the cause of spinocerebellar ataxia type 7 (SCA7); also known as olivopontocerebellar atrophy III (OPCA III or OPCA3) or olivopontocerebellar atrophy with retinal degeneration. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA7 belongs to the autosomal dominant cerebellar ataxias type II (ADCA II) which are characterized by cerebellar ataxia with retinal degeneration and pigmentary macular dystrophy. Belongs to the ataxin-7 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nucleolus; Transcription regulation

Chromosomal Location of Human Ortholog: 3p21.1-p12

Cellular Component: nucleoplasm; microtubule cytoskeleton; nuclear matrix; cytoplasm; nucleolus; nucleus

Molecular Function: protein binding; chromatin binding

Biological Process: establishment and/or maintenance of chromatin architecture; visual perception; transcription, DNA-dependent; negative regulation of insulin-like growth factor receptor signaling pathway; negative regulation of phosphorylation; positive regulation of transcription from RNA polymerase II promoter; microtubule cytoskeleton organization and biogenesis; nuclear organization and biogenesis; histone deubiquitination

Disease: Spinocerebellar Ataxia 7