Skeletal muscle
- Neuromuscular disease modeling on a chip
Summary: Modeling neuromuscular diseases is challenging due to their complex etiology and pathophysiology. Here, we review the cell sources and tissue-engineering procedures that are being integrated as emerging neuromuscular disease models.
- HCT116 colorectal liver metastases exacerbate muscle wasting in a mouse model for the study of colorectal cancer cachexia
Summary: Colorectal cancer-derived liver metastases exacerbate cachexia, in line with activation of STAT3 signaling. STAT3 inhibition may improve cancer-associated muscle wasting.
- Cored in the act: the use of models to understand core myopathies
Summary: The core myopathies are neuromuscular disorders with no cure. In this Review, we outline our current understanding of pathomechanisms, the advantages and limitations of available models, and discuss emerging modeling technologies.
- C9ORF72-related cellular pathology in skeletal myocytes derived from ALS-patient induced pluripotent stem cells
Summary: Evidence of protein aggregation and mitochondrial dysfunction were found in skeletal myocytes differentiated from ALS-patient induced pluripotent stem cells with the C9ORF72 mutation.
- Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease
Summary: Specific mitochondrial parameters in muscle of a minipig model of Huntington's disease can be used as potential biomarkers of disease progression and suggest that similar pathogenic mechanisms underlie disease progression in TgHD minipigs and HD patients.
- Therapeutic strategies for spinal muscular atrophy: SMN and beyond
Summary: Translational research for spinal muscular atrophy (SMA) should address the development of non-CNS and survival motor neuron (SMN)-independent therapeutic approaches to complement and enhance the benefits of CNS-directed and SMN-dependent therapies.
- Poor maternal nutrition and accelerated postnatal growth induces an accelerated aging phenotype and oxidative stress in skeletal muscle of male rats
Summary: Muscle of ‘developmentally programmed’ rat offspring demonstrated accelerated aging and oxidative stress, which could explain why some individuals are at greater risk of developing age-associated muscular dysfunction than others.
- Angiotensin-(1-7) attenuates disuse skeletal muscle atrophy in mice via its receptor, Mas
Summary: In this article, the authors demonstrate that a peptide with actions mainly in the cardiovascular system prevents the skeletal muscle damage induced by disuse.
- The role of mTOR signaling in the regulation of protein synthesis and muscle mass during immobilization in mice
Summary: The activation of mTOR signaling is both necessary and sufficient to alleviate the decreases in protein synthesis and muscle mass that occur during immobilization.
- Phospholamban overexpression in mice causes a centronuclear myopathy-like phenotype
Summary: Phospholamban overexpression in mouse slow-twitch muscle impairs SERCA function and causes histopathological features associated with human centronuclear myopathy.