Abnormal DNA Repair Mechanism May Be Therapeutic Target

Abnormal DNA Repair Mechanism May Be Therapeutic Target

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Abnormal DNA Repair Mechanism May Be Therapeutic Target

The interaction between FAN1 and MLH1 — two DNA repair proteins known to be genetic modifiers of Huntington’s disease — protects against further expansion of disease-causing CAG repeats, according to a study using human and mouse models of the disease. Specifically, this interaction prevents MLH1’s recruitment to a DNA repair protein complex that, when bound to MLH1, abnormally promotes CAG repeat expansion. FAN1 itself also was found to contribute to repeat stabilization. “Evidence for DNA…

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