Rabbit Ataxin 7 Polyclonal Antibody | anti-Ataxin7 antibody

Ataxin 7 Polyclonal Antibody

Cat. Num. AAA3015216

• Gene Names: ATXN7; SCA7; OPCA3; ADCAII; FLJ17787
• Reactivity: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep
• Applications: Immunohistochemistry
• Purity: affinity purified by Protein A
• Synonyms: Ataxin 7; Polyclonal Antibody; Ataxin 7 Polyclonal Antibody; Ataxin7; Ataxin-7; ADCAII; ATXN 7; OPCA III; OPCA3; SCA 7; SCA7; Spinocerebellar Ataxia 7; Spinocerebellar ataxia type 7 protein; ATX7_HUMAN; anti-Ataxin7 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep
• Clonality: Polyclonal
• Specificity: Ataxin 7 Polyclonal Antibody detects endogenous levels of Ataxin 7 protein.
• Purity/Purification: affinity purified by Protein A
• Form/Format: 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

• Applicable Applications for anti-Ataxin7 antibody: Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: IHC-P: 1:100-500
• Immunogen: KLH conjugated synthetic peptide derived from human Ataxin 7
• Preparation and Storage: Store at 4 degree C short term. Aliquot and store at -20 degree C long term. Avoid freeze-thaw cycles.

Immunohistochemistry (IHC)

(Paraformaldehyde-fixed, paraffin embedded (mouse cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Ataxin 7) Polyclonal Antibody, Unconjugated at 1:200 overnight at 4°C)

Related Product Information for anti-Ataxin7 antibody

The human ataxin-7 gene, also known as spinocerebellar ataxia 7 or SCA7, maps to chromosome 3p13-p12, has a 2,727-bp open reading frame, and encodes a 892 amino acid protein containing a nuclear localization signal and a polyglutamine tract (1,2). SCA7 is an autosomal dominant neurodegenerative disorder characterized by ataxia and selective neuronal cell loss caused by the expansion of a translated CAG repeat encoding a polyglutamine tract in ataxin-7, which is the SCA7 gene product (3,4). Ataxin-7 is expressed within neurons both affected and unaffected in SCA7 pathology with subcellular localization being variable depending upon the neuronal subtype (5). Polyglutamine expanded in ataxin-7 may carry out its pathogenic effects in the nucleus by altering the matrix-associated nuclear structure and/or by disrupting nucleolar function (6).

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
• 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