Background:

Autosomal dominant cerebellar ataxias are a group of neurodegenerative disorders caused by unstable CAG repeat expansions encoding polyglutamine tracts. Proteins with long polyglutamine tracts have an increased tendency to aggregate, often forming ubiquitinated intranuclear inclusion bodies. Ataxin-2, the gene product of the human spinocerebellar ataxia type 2 (SCA2/ATXN2) gene, is a basic protein with two domains (Sm1 and Sm2) implicated in RNA splicing and protein interaction. Ataxin-2 interacts with a putative RNA-binding protein ataxin-2-binding-protein 1 (A2BP1), which is expressed in muscle and brain. Ataxin-2 is ubiquitously expressed with highest levels in the cytoplasm of Purkinje cells. Both A2BP1 and ataxin-2 are localized to the trans-Golgi network. Mice expressing ataxin-2 with polyglutamine show progressive functional deficits accompanied by loss of Purkinje cell dendritic arbor and eventually loss of Purkinje cells. In conclusion, expansion of ataxin-2 results in spinocerebellar ataxia type 2, which affects the cerebellum and other areas of the central nervous system.

Alternative names:

ATXN2; ATX2; SCA2; TNRC13; Ataxin-2; Spinocerebellar ataxia type 2 protein; Trinucleotide repeat-containing gene 13 protein

Clonality:

Polyclonal

Reactivity:

Human

Source:

Rabbit

Isotype:

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Immunogen:

Synthesized peptide derived from the Internal region of human Ataxin-2. at AA rangle； 700-780

Concentration:

1mg/ml

Applications:

WB, IHC, ELISA

Recommende dilutions:

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Storage:

PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.

More info:

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