Defective regulation of MicroRNA target genes in myoblasts from facioscapulohumeral dystrophy patients

Abstract

Background: FSHD is characterized by the overexpression of double homeobox genes DUX4 and DUX4c. Results: We found 29 miRNAs differentially expressed between FSHD and normal myoblasts. Twelve of these miRNAs were up-regulated in myoblasts ectopically expressing DUX4c. Conclusion: DUX4c is linked to the abnormal miRNA expression profile observed in FSHD. Significance: We observe a defective gene regulation by miRNAs in FSHD. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc.