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The SETX gene provides the chemical instructions for making a high-molecular-weight protein called senataxin. Produced in a wide range of tissues—including the brain, spinal cord, and muscles—senataxin functions as a cellular “guardian” that maintains genomic stability and ensures proper gene expression. How the SETX Gene Works

Senataxin belongs to a class of proteins called helicases, which are molecular motors that bind to DNA or RNA and temporarily unwind their strands. By doing so, senataxin orchestrates several critical cellular processes: SETX gene: MedlinePlus Genetics

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