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| Product Name | ATXN1 [4C7B11] |
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| Antibody Type | Primary Antibodies |
| Product 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. |
| Immunogen | Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli. |
| Clonality | Monoclonal |
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| Isotype | IgG1 |
| Host Species | Mouse |
| Tested Applications | WB,IHC,FC |
| WB:1:500-1:2,000 IHC:1:50-1:200 FC:1:100-1:200 | |
| Species Reactivity | Human,Mouse,Rat,Monkey |
| Concentration | 1mg/mL |
| Purification | Unpurified |
| Alternative Names | alternative ataxin1 antibody Ataxin-1 antibody ATX1 antibody ATX1_HUMAN antibody Atxn1 antibody D6S504E antibody OTTHUMP00000016065 antibody SCA1 antibody Spinocerebellar ataxia type 1 protein antibody |
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| Molecular Weight(MW) | 86.9kDa |
| Cellular Localization | Cytoplasm. Nucleus. Colocalizes with USP7 in the nucleus. |
| SwissProt ID | P54253 |
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Application
Western blot analysis of ATXN1 against human ATXN1 (AA: 645-815) recombinant protein. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (EM1711-76, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.
Application
Western blot analysis of ATXN1 against HEK293 (1) and ATXN1 (AA: 645-815)-hIgGFc transfected HEK293 (2) cell lysate. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (EM1711-76, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.
Application
Western blot analysis of ATXN1 against C6 (1), COS7 (2), NIH/3T3 (3), and HL-60 (4) cell lysate. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (EM1711-76, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Mouse IgG - HRP Secondary Antibody at 1:5,000 dilution was used for 1 hour at room temperature.
Application
Immunohistochemical analysis of paraffin-embedded endometrial cancer tissues using anti-ATXN1 antibody. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 8.0) for 20 minutes. The tissues were blocked in 5% BSA for 30 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (EM1711-76, 1/100) for 30 minutes at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Application
Flow cytometric analysis of ATXN1 was done on Jurkat cells . The cells were fixed, permeabilized and stained with the primary antibody (EM1711-76, 1/100) (green). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated goat anti-Mouse IgG Secondary antibody at 1/500 dilution for 30 minutes. Unlabelled sample was used as a control (cells without incubation with primary antibody; red).| Positive Control | C6, COS7, NIH/3T3, and HL-60 cell lysate, Jurkat cells, endometrial cancer tissues |
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| Application Notes | WB:1:500-1:2,000 IHC:1:50-1:200 FC:1:100-1:200 |
| Form | Liquid |
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| Storage Instructions | Store at +4℃ after thawing. Aliquot store at -20℃. Avoid repeated freeze / thaw cycles. |
| Storage Buffer | 1*PBS with 0.05% sodium azide. |
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