Pathophysiological roles of DUX4 in FSHD1 (Homo sapiens)

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This pathway shows how the aberrant expression of DUX4 in post-embryonic stages leads to several disturbances in skeletal muscle fibers, causing inflammation, apoptosis and other processes contributing to Facioscapulohumeral muscular dystrophy (FSHD).</Comment> <Comment Source="WikiPathways-description">DUX4 downstream signaling in FSHD. Low D4Z4 subunit methylation, which is caused either by low amount of D4Z4 subunits combined with a permissive 4q haplotype, or a mutation in genes involved with D4Z4 methylation, results in the expression of DUX4. Normally, DUX4 is only expressed during embryogenesis, after which it is silenced. 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