Deregulation of IGF2BP1-mRNP components during the differentiation of FSHD muscle cells


Facioscapulohumeral muscular dystrophy (FSHD) is caused by the misexpression of DUX4. Its homologue DUX4c is also induced in FSHD muscles. In contrast to DUX4, DUX4c is expressed in all myoblasts and is proposed to play a role in normal muscle regeneration. DUX proteins are transcription factors but their normal functions in muscle are not understood yet. In FSHD muscle fibers, myofibril disorganization and sarcomeric dysfunction are shown but are not explained until now by the DUX4 target genes.


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