Biallelic variants in GTPBP3: New patients, phenotypic spectrum, and outcome.
Francesca NardecchiaRosalba CarrozzoAlice InnocentiAlessandra TorracoValerio ZaccariaTeresa RizzaFrancesco PisaniEnrico BertiniVincenzo LeuzziPublished in: Annals of clinical and translational neurology (2024)
Reviewed cases point toward clustering around two prevalent phenotypes: an early-onset presentation with severe fatal encephalopathy and a late milder presentation with global developmental delay/ID and cardiopathy, with the latter as, is the main feature. Our patients showed an intermediate phenotype with intrafamilial variability.