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Description

FRDA, Friedreich ataxia

As the most prevalent inhibitory neurotransmitter, GABA exerts its effects by reducing neuronal excitability, thus preventing excessive neuronal firing and maintaining the stability of neural circuits

The 3 children with MD that died (aged 66192 months) while being on KD all had POLG- related Alpers disease, an early lethal disorder with a median age at death of 16 (range 1181) months [48]