Incidence of 1 in 10,000 to 1 in 20,000 [MISCELLANEOUS] 70% due to de novo maternal deletion of 15q11.2-q13 [MISCELLANEOUS] 2-3% due to imprinting defects [MISCELLANEOUS] Increased sensitivity to heat [MISCELLANEOUS] Caused by mutation in the ubiquitin protein ligase E3A gene (UBE3A, 601623.0001) [MOLECULAR BASIS] Onset between 6 and 12 months of age [MISCELLANEOUS] Imprinted disorder [MISCELLANEOUS] 25% due to mutations in UBE3A (601623) [MISCELLANEOUS] 2% due to paternal uniparental disomy of 15q11.2-q13 [MISCELLANEOUS]
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