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Ataxin 7 Rabbit pAb
Ataxin 7 Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application 
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | O15265 |
| Reactivity | Mouse, Rat |
| Predicted | Human, Dog, Pig, Horse, Rabbit, Sheep |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 95451 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human Ataxin 7 |
| Epitope Specificity | 301-400/892 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Cytoplasmic (isoform b) and Nuclear (isoform a) |
| SIMILARITY | Belongs to the ataxin-7 family. Contains 1 SCA7 domain. |
| SUBUNIT | Component of the STAGA transcription coactivator-HAT complex, at least composed of SUPT3H, GCN5L2, TAF5L, TAF6L, SUPT7L, TADA3L, TAD1L, TAF10, TAF12, TRRAP, TAF9 and ATXN7. The STAGA core complex is associated with a subcomplex required for histone deubiquitination composed of ATXN7L3, ENY2 and USP22. Interacts with SORBS1, PSMC1 and CRX. Interacts with TRRAP, GCN5L2 and TAF10. Interacts with alpha tubulin. |
| Post-translational modifications | Proteolytically cleaved. The cleavage may be involved in SCA7 degeneration: the isoform fragments may exert distinct toxic influences that could contribute to selective neurodegeneration.Sumoylation decreases the aggregation propensity and cellular toxicity of forms with an expanded poly-Gln region but has no effect on subcellular location or interaction with components of the STAGA complex. |
| DISEASE | Defects in ATXN7 are the cause of spinocerebellar ataxia type 7 (SCA7) [MIM:164500]; 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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). |
| Gene ID | 6314 |
|---|---|
| Other Names | Ataxin-7, Spinocerebellar ataxia type 7 protein, ATXN7, SCA7 {ECO:0000303|PubMed:9288099} |
| Target/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, lymphoblast and intestine. |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| Name | ATXN7 |
|---|---|
| Synonyms | SCA7 {ECO:0000303|PubMed:9288099} |
| Function | Acts as a component of the SAGA (aka STAGA) transcription coactivator-HAT complex (PubMed:15932940, PubMed:18206972). Mediates the interaction of SAGA complex with the CRX and is involved in CRX- dependent gene activation (PubMed:15932940, PubMed:18206972). Probably involved in tethering the deubiquitination module within the SAGA complex (PubMed:24493646). Necessary for microtubule cytoskeleton stabilization (PubMed:22100762). Involved in neurodegeneration (PubMed:9288099). |
| Cellular 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 (PubMed:10441328). It is able to shuttle between the nucleus and cytoplasm (PubMed:16314424) |
| Tissue Location | [Isoform a]: Isoform a is expressed in CNS, but is expressed predominantly in the peripherical tissues |
Research Areas
For Research Use Only. Not For Use In Diagnostic Procedures.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
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).
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