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您的检索式：作者名="Frederic Relaix"
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| 1 | Master regulators of skeletal muscle lineage development and pluripotent stem cells differentiation显示文摘In vertebrates, the skeletal muscles of the body and their associated stem cells originate from muscle progenitor cells,during development. The specification of the muscles of the trunk, head and limbs, relies on the activity of distinctgenetic hierarchies. The major regulators of trunk and limb muscle specification are the paired-homeobox transcriptionfactors PAX3 and PAX7. Distinct gene regulatory networks drive the formation of the different muscles of thehead. Despite the redeployment of diverse upstream regulators of muscle progenitor differentiation, the commitmenttowards the myogenic fate requires the expression of the early myogenic regulatory factors MYF5, MRF4, MYOD andthe late differentiation marker MYOG. The expression of these genes is activated by muscle progenitors throughoutdevelopment, in several waves of myogenic differentiation, constituting the embryonic, fetal and postnatal phases ofmuscle growth. In order to achieve myogenic cell commitment while maintaining an undifferentiated pool of muscleprogenitors, several signaling pathways regulate the switch between proliferation and differentiation of myoblasts.The identification of the gene regulatory networks operating during myogenesis is crucial for the development ofin vitro protocols to differentiate pluripotent stem cells into myoblasts required for regenerative medicine. | Joana Esteves de Lima Frederic Relaix | 2021 | Cell Regeneration2021，10，1： | 1 |
| 2 | HACD1,a regulator of membrane composition and fluidity,promotes myoblast fusion and skeletal muscle growth显示文摘The reduced diameter of skeletal myofibres is a hallmark of several congenital myopathies,yet the underlying cellular and molecular mechanisms remain elusive.In this study,we investigate the role of HACD1/PTPLA,which is involved in the elongation of the very long chain fatty acids,in muscle fibre formation.In humans and dogs,HACD1 deficiency leads to a congenital myopathy with fibre size disproportion associated with a generalized muscleweakness.Throughanalysis of HACD1-deficient Labradors,Hacd1-knockout mice,and Hacd1-deficient myoblasts,we provide evidence that HACD1 promotes myoblast fusion during muscle development and regeneration.We further demonstrate that in normal differentiating myoblasts,expression of the catalytically active HACD1 isoform,which is encoded by a muscle-enriched splice variant,yields decreased lysophosphatidylcholine content,a potent inhibitor of myoblast fusion,and increased concentrations of≥C18 and monounsaturated fatty acids of phospholipids.These lipid modifications correlate with a reduction in plasma membrane rigidity.In conclusion,we propose that fusion impairment constitutes a novel,non-exclusive pathological mechanism operating in congenital myopathies and reveal that HACD1 is a key regulator of a lipid-dependent muscle fibre growth mechanism. | Jordan Blondelle Yusuke Ohno Vincent Gache Stephane Guyot Sebastien Storck Nicolas Blanchard-Gutton Ines Barthelemy Gemma Walmsley Anaelle Rahier Stephanie Gadin Marie Maurer Laurent Guillaud Alexandre Prola Arnaud Ferry Genevieve Aubin-Houzelstein Jean Demarquoy Frederic Relaix Richard JPiercy Stephane Blot Akio Kihara Laurent Tiret Fanny Pilot-Storck | 2015 | Journal of Molecular Cell Biology2015，7，5： | 1 |
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