Reactivity: | H & M,R |
Applications: | WB & IHC/IF |
Host Species: | Rabbit |
Clonality: | Polyclonal |
Full Name: | Ataxin 1 rabbit polyclonal antibody |
Gene Name: | Ataxin-1 |
Synonyms: | Spinocerebellar ataxia type 1 protein homolog, Atxn1, Sca1, SCA1D6S504E |
Immunogen: | Recombinant protein corresponding to Mouse Ataxin 1 |
Isotype: | IgG |
Purity: | Affinity purification |
Subcellular location: | Cytoplasm, Nucleus |
Predicted MW. | 84 kDa |
Observed MW. | 100 kDa |
Uniprot ID: | P54254, Q63540 |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
WB | Human | 1: 500-1: 1000 | SH-SY5Y, U87, U-251MG |
IHC/IF | Mouse, Rat | 1: 500-1: 3000 | fetal brain, lung |
Background
Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) ; also known as olivopontocerebellar atrophy I(OPCA I or OPCA1). 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 cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I(ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia.
Images
Western blot analysis of ataxin1 (GB11988) at dilution of 1: 500 |
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Immunohistochemistry of paraffin embedded mouse fetal brain using ataxin1 (GB11988) at dilution of 1: 1800 (300x lens) |
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Immunohistochemistry of paraffin embedded mouse lung using ataxin1 (GB11988) at dilution of 1: 1800 (250x lens) |
Storage
Storage | Store at -20°C for one year. Avoid repeated freeze/ thaw cycles. |
Storage Buffer | PBS with 0.02% sodium azide, 100 μg/ml BSA and 50% glycerol. |