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Table 1 FSHD-like mouse severity models

From: Transgenic mice expressing tunable levels of DUX4 develop characteristic facioscapulohumeral muscular dystrophy-like pathophysiology ranging in severity

GenotypeTMXTransgene recombinationDUX4-fl expressionPhenotype
ACTA1-MCM/+5 mg/kg × 1 IPNANoControl moderate
ACTA1-MCM/+10 mg/kg × 2 IPNANoControl Severe
ACTA1-MCM;FLExD/+None2-10%YesMild
ACTA1-MCM;FLExD/+5 mg/kg × 1 IP7–28%YesModerate
ACTA1-MCM;FLExD/+10 mg/kg × 2 IP38–55%YesSevere