Ataxin

• Ataxin 1, coded by ATXN1. Mutants of ataxin 1 with a polyglutamine expansion cause SCA1.
• Ataxin 2, coded by ATXN2. It is known to cause SCA2 with polyglutamine expansion.
• Ataxin 3, coded by ATXN3. Machado-Joseph disease is caused by polyglutamine expansions in ataxin 3.
• Ataxin 7, coded by ATXN7. Polyglutamine expansions in Ataxin 7 cause SCA7.
• Ataxin 8, coded by ATXN8. Ataxin 8 does not cause an ataxic order, but a gene on the opposite strand, ATXN8OS, causes Spinocerebellar ataxia type 8 with CTG expansion.
• Ataxin 10, coded by ATXN10. It is associated with the pentanucleotide disorder, SCA10.
• Frataxin, follows a similar naming convention and is coded by the FXN gene. GAA repeat expansions in a non-coding region of FXN cause Friedreich's ataxia when both copies of the gene are affected.