The role of ZC3H14 (MSUT2) in neurodevelopment and tauopathies.

ZC3H14 (MSUT2) loss-of-function is linked to inherited intellectual disability and, in model systems, can suppress tau aggregate toxicity in tauopathies.

• Why it matters: Neurodevelopment and neuropathology are shaped by RNA binding proteins, but how ZC3H14 dysfunction connects cognitive outcomes to tau pathology remains unclear.
• What they did: This review synthesizes evidence on ZC3H14’s normal roles and on how its altered RNA regulation may affect neuronal function and tau pathology.
• The result: The emerging theme is that ZC3H14-related changes in RNA regulation can influence neuronal function, and model-system findings suggest modulation of tau aggregate toxicity.

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