Description
Ataxin 3 is also known as Machado-Joseph disease protein 1. Machado–Joseph disease is one of several hereditary autosomal dominant neurodegenerative disorders. This protein contains trinucleotide CAG repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. Ataxin 3 interacts with the major histone acetyltransferases cAMP-response-element binding protein (CREB)-binding protein, p300, and p300/CREB-binding protein-associated factor and inhibits transcription by these coactivators. Also, ataxin-3 is a proteasome-associated factor that mediates the degradation of ubiquitinated proteins