Search Results - "Castets, Perrine"
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Sustained Activation of mTORC1 in Skeletal Muscle Inhibits Constitutive and Starvation-Induced Autophagy and Causes a Severe, Late-Onset Myopathy
Published in Cell metabolism (07-05-2013)“…Autophagy is a catabolic process that ensures homeostatic cell clearance and is deregulated in a growing number of myopathological conditions. Although FoxO3…”
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2
Rapamycin attenuates the progression of tau pathology in P301S tau transgenic mice
Published in PloS one (07-05-2013)“…Altered autophagy contributes to the pathogenesis of Alzheimer's disease and other tauopathies, for which curative treatment options are still lacking. We have…”
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mTORC1 and PKB/Akt control the muscle response to denervation by regulating autophagy and HDAC4
Published in Nature communications (18-07-2019)“…Loss of innervation of skeletal muscle is a determinant event in several muscle diseases. Although several effectors have been identified, the pathways…”
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Targeting deregulated AMPK/mTORC1 pathways improves muscle function in myotonic dystrophy type I
Published in The Journal of clinical investigation (01-02-2017)“…Myotonic dystrophy type I (DM1) is a disabling multisystemic disease that predominantly affects skeletal muscle. It is caused by expanded CTG repeats in the…”
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Activation of mTORC1 in skeletal muscle regulates whole-body metabolism through FGF21
Published in Science signaling (10-11-2015)“…Skeletal muscle is the largest organ, comprising 40% of the total body lean mass, and affects whole-body metabolism in multiple ways. We investigated the…”
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The TOR Pathway at the Neuromuscular Junction: More Than a Metabolic Player?
Published in Frontiers in molecular neuroscience (28-08-2020)“…The neuromuscular junction (NMJ) is the chemical synapse connecting motor neurons and skeletal muscle fibers. NMJs allow all voluntary movements, and ensure…”
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CaMKIIβ deregulation contributes to neuromuscular junction destabilization in Myotonic Dystrophy type I
Published in Skeletal muscle (21-05-2024)“…Myotonic Dystrophy type I (DM1) is the most common muscular dystrophy in adults. Previous reports have highlighted that neuromuscular junctions (NMJs)…”
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Satellite cell loss and impaired muscle regeneration in selenoprotein N deficiency
Published in Human molecular genetics (15-02-2011)“…Selenoprotein N (SelN) deficiency causes a group of inherited neuromuscular disorders termed SEPN1-related myopathies (SEPN1-RM). Although the function of SelN…”
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Increased muscle stress-sensitivity induced by selenoprotein N inactivation in mouse: a mammalian model for SEPN1-related myopathy
Published in PloS one (08-08-2011)“…Selenium is an essential trace element and selenoprotein N (SelN) was the first selenium-containing protein shown to be directly involved in human inherited…”
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10
mTORC2 affects the maintenance of the muscle stem cell pool
Published in Skeletal muscle (02-12-2019)“…The mammalian target of rapamycin complex 2 (mTORC2), containing the essential protein rictor, regulates cellular metabolism and cytoskeletal organization by…”
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Differential response of skeletal muscles to mTORC1 signaling during atrophy and hypertrophy
Published in Skeletal muscle (06-03-2013)“…Skeletal muscle mass is determined by the balance between protein synthesis and degradation. Mammalian target of rapamycin complex 1 (mTORC1) is a master…”
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12
"Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular Disorders
Published in Journal of neuromuscular diseases (27-05-2016)“…Recent research has revealed that autophagy, a major catabolic process in cells, is dysregulated in several neuromuscular diseases and contributes to the…”
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13
Back to basics: Optimization of DNA and RNA transfer in muscle cells using recent transfection reagents
Published in Experimental cell research (15-12-2022)“…C2C12 cells are widely used in the muscle field, as they differentiate easily into myotubes and show limited constraints to culture as compared to primary…”
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14
mTOR controls embryonic and adult myogenesis via mTORC1
Published in Development (Cambridge) (01-04-2019)“…The formation of multi-nucleated muscle fibers from progenitors requires the fine-tuned and coordinated regulation of proliferation, differentiation and…”
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Selenoprotein N in skeletal muscle: from diseases to function
Published in Journal of molecular medicine (Berlin, Germany) (01-10-2012)“…Selenoprotein N (SelN) deficiency causes several inherited neuromuscular disorders collectively termed SEPN1 -related myopathies, characterized by early onset,…”
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Identification of a muscle-specific isoform of VMA21 as a potent actor in X-linked myopathy with excessive autophagy pathogenesis
Published in Human molecular genetics (01-12-2023)“…Abstract Defective lysosomal acidification is responsible for a large range of multi-systemic disorders associated with impaired autophagy. Diseases caused by…”
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MTORC1 determines autophagy through ULK1 regulation in skeletal muscle
Published in Autophagy (01-09-2013)“…Autophagy impairment has been implicated in several muscle disorders and in age-related dysfunction. Although previous reports pointed to FOXO as a positive…”
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CaMKII[beta] deregulation contributes to neuromuscular junction destabilization in Myotonic Dystrophy type I
Published in Skeletal muscle (21-05-2024)“…Myotonic Dystrophy type I (DM1) is the most common muscular dystrophy in adults. Previous reports have highlighted that neuromuscular junctions (NMJs)…”
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Mutations in the selenocysteine insertion sequence-binding protein 2 gene lead to a multisystem selenoprotein deficiency disorder in humans
Published in The Journal of clinical investigation (01-12-2010)“…Selenium, a trace element that is fundamental to human health, is incorporated into some proteins as selenocysteine (Sec), generating a family of…”
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Selenoprotein N is dynamically expressed during mouse development and detected early in muscle precursors
Published in BMC developmental biology (22-08-2009)“…In humans, mutations in the SEPN1 gene, encoding selenoprotein N (SelN), are involved in early onset recessive neuromuscular disorders, referred to as…”
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