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A group of proteins that are defective in various forms of spinocerebellar ataxia (SCA) because of expanded numbers of trinucleotide (CAG) repeats (polyglutamine repeats). Ataxin 1 (815 aa), defective in SCA1, binds RNA and may be involved in RNA metabolism. Ataxin 2 (1313 aa) is defective in SCA2. Ataxin 3 (Machado–Joseph disease protein 1, 376 aa) defective in SCA3, represses transcription by interacting with transcriptional regulators and binding to histones. Ataxin 7 (892 aa), defective in SCA7, is a component of the STAGA transcription coactivator-HAT complex. Ataxin 10 (475 aa) is defective in SCA10. Ataxin 1-like (brother of ataxin-1, BOAT, 689 aa) will suppress the cytotoxicity of ataxin 1 in SCA1. Ataxin-7-like protein 1 (833 aa) is known, but not its function. Ataxin-7-like protein 3 (347 aa) is part of the SAGA transcription regulatory complex.

Subjects: Chemistry — Medicine and Health.

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