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Electroencephalography (EEG) and related methodologies have emerged as powerful tools in both, clinical and preclinical research programs, for the identification of physiological and pathological biomarkers. Nowadays, EEG endpoints are commonly used in the clinic to identify and evaluate a pathological state and/or as surrogate biomarkers for clinical trials. In this work, we took advantage of quantitative electroencephalography (qEEG) to characterize a humanized transgenic mouse line carrying a mutation in the GABA-A signaling, a mutation observed in patients with rare diseases.