Senataxin Proteins and Enzymes

Mutations in Senataxin result in the neurodegenerative disorders Ataxia-Oculomotor Apraxia 2 (AOA2) and amyotrophic lateral sclerosis (ALS4). Senataxin bears an RNA/DNA helicase domain suggesting a role in DNA and RNA processing and has recently been found to be involved in the DNA damage response. Alternate names for Senataxin include SEN1 homolog, SETX, ALS4, KIAA0625, SCAR1, and AOA2.