SETX


Probable helicase senataxin is an enzyme that in humans is encoded by the SETX gene.
This gene encodes a protein named for its homology to the Sen1p protein of fungi which has RNA helicase activity encoded by a domain at the C-terminal end of the protein. The protein encoded by this gene contains a DNA/RNA helicase domain at its C-terminal end which suggests that it may be involved in both DNA and RNA processing. Mutations in this gene have been associated with Ataxia oculomotor apraxia type 2 and an autosomal dominant form of juvenile amyotrophic lateral sclerosis.