Brain & development · 2026
Elevated delta power in a maternal UBE3A-deletion pig model of Angelman syndrome.
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Myers Luke S, Monk Tanner G, Martinez Luis A, Taylor Alasdair J, Christian Sarah G, Anderson Anne E, Dindot Scott V
Аннотация
BACKGROUND: Angelman syndrome is a neurodevelopmental disorder caused by loss of the maternally inherited UBE3A allele and characterized by severe cognitive, motor, and communication impairments. Increased delta (1-4 Hz) activity on electroencephalogram (EEG) is a well-established biomarker of Angelman syndrome in humans and rodent models and is increasingly used as a quantitative outcome measure in therapeutic studies. Whether this electrophysiological phenotype is present in the recently developed pig model has not been evaluated.
METHODS: Scalp EEG recordings were obtained from freely moving Yorkshire-Landrace pigs carrying a maternal UBE3A deletion (UBE3A-/+) and wild-type littermates across three developmental age groups. Artifact-free EEG epochs were identified, behavioral states were classified using synchronized video, and relative delta power was quantified using spectral analysis.
RESULTS: UBE3A-/+ pigs showed higher relative delta power than wild-type pigs during both awake and asleep vigilance states, with the largest genotype differences observed during wakefulness. Across development, UBE3A-/+ pigs also showed a smaller increase in delta power during the transition from the awake to the asleep state compared with wild-type pigs.
CONCLUSIONS: These findings demonstrate that the maternal UBE3A-deletion pig model reproduces the elevated delta power EEG phenotype characteristic of Angelman syndrome. Noninvasive scalp EEG therefore represents a translationally relevant approach for assessing neural dysfunction and therapeutic responses in this large-animal model.
METHODS: Scalp EEG recordings were obtained from freely moving Yorkshire-Landrace pigs carrying a maternal UBE3A deletion (UBE3A-/+) and wild-type littermates across three developmental age groups. Artifact-free EEG epochs were identified, behavioral states were classified using synchronized video, and relative delta power was quantified using spectral analysis.
RESULTS: UBE3A-/+ pigs showed higher relative delta power than wild-type pigs during both awake and asleep vigilance states, with the largest genotype differences observed during wakefulness. Across development, UBE3A-/+ pigs also showed a smaller increase in delta power during the transition from the awake to the asleep state compared with wild-type pigs.
CONCLUSIONS: These findings demonstrate that the maternal UBE3A-deletion pig model reproduces the elevated delta power EEG phenotype characteristic of Angelman syndrome. Noninvasive scalp EEG therefore represents a translationally relevant approach for assessing neural dysfunction and therapeutic responses in this large-animal model.