inositol-1-4-5-trisphosphate and Muscular-Dystrophy--Duchenne

inositol-1-4-5-trisphosphate has been researched along with Muscular-Dystrophy--Duchenne* in 1 studies

Other Studies

1 other study(ies) available for inositol-1-4-5-trisphosphate and Muscular-Dystrophy--Duchenne

ArticleYear
Inositol 1,4,5-trisphosphate (IP3)-dependent Ca2+ signaling mediates delayed myogenesis in Duchenne muscular dystrophy fetal muscle.
    Development (Cambridge, England), 2016, Feb-15, Volume: 143, Issue:4

    Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disorder characterized by muscle wasting and premature death. The defective gene is dystrophin, a structural protein, absence of which causes membrane fragility and myofiber necrosis. Several lines of evidence showed that in adult DMD patients dystrophin is involved in signaling pathways that regulate calcium homeostasis and differentiation programs. However, secondary aspects of the disease, such as inflammation and fibrosis development, might represent a bias in the analysis. Because fetal muscle is not influenced by gravity and does not suffer from mechanical load and/or inflammation, we investigated 12-week-old fetal DMD skeletal muscles, highlighting for the first time early alterations in signaling pathways mediated by the absence of dystrophin itself. We found that PLC/IP3/IP3R/Ryr1/Ca(2+) signaling is widely active in fetal DMD skeletal muscles and, through the calcium-dependent PKCĪ± protein, exerts a fundamental regulatory role in delaying myogenesis and in myofiber commitment. These data provide new insights into the origin of DMD pathology during muscle development.

    Topics: Animals; Biomarkers; Biopsy; Calcium; Calcium Channels; Calcium Signaling; Fetus; Gene Expression Regulation, Developmental; Inositol 1,4,5-Trisphosphate; Mice, Inbred C57BL; Mice, Inbred mdx; Models, Biological; Muscle Development; Muscle Fibers, Skeletal; Muscle, Skeletal; Muscular Dystrophy, Animal; Muscular Dystrophy, Duchenne; PAX7 Transcription Factor; Protein Kinase C-alpha

2016