Premium
Dux4 target gene expression in mouse muscle transplanted with muscle cells from FSHD patients
Author(s) -
Windelborn James,
Rahimov Fedik,
Emerson Charles P
Publication year - 2013
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.27.1_supplement.751.6
Subject(s) - myocyte , pathogenesis , tibialis anterior muscle , biology , gene , skeletal muscle , cancer research , pathology , medicine , microbiology and biotechnology , anatomy , genetics
In order to gain insights into FSHD molecular pathology, we developed a xenograft model by transplanting myogenic cells from patients with FSHD (4qA contractions) as well as from their unaffected relatives into the tibialis anterior muscles of immunodeficient mice. Our findings show that muscle xenografts derived from FSHD myogenic cells express Dux4 target genes, recapitulating the expression of these disease biomarkers in muscle biopsies of FSHD patients. FSHD muscle xenografts provide an animal model for investigations of the molecular pathogenesis of FSHD muscles and for drug development. Grant Funding Source : NIH 5U54HD06084805 and FSH Society FSHS‐22012–04