Article
Neurosciences
Elana Molotsky, Yuhong Liu, Andrew P. Lieberman, Diane E. Merry
Summary: This study examines the relationship between neuromuscular junction (NMJ) pathology and vulnerability of fast-twitch motor units in spinal and bulbar muscular atrophy (SBMA) mouse models. The research reveals significantly increased NMJ and myofiber pathology in fast-twitch motor units, along with metabolic dysregulation and myofiber atrophy.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2022)
Article
Geriatrics & Gerontology
Anna Carolina Zaia Rodrigues, Zhong-Min Wang, Maria Laura Messi, Henry Jacob Bonilla, Liang Liu, Willard M. Freeman, Osvaldo Delbono
Summary: The study found that inducing the expression of Hand2 in sympathetic neurons in old mice can increase neuron size and number, improve muscle weight and force, enhance muscle innervation, promote muscle transmission, prevent inflammation, and maintain muscle protein synthesis.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Article
Geriatrics & Gerontology
Anna Carolina Zaia Rodrigues, Maria Laura Messi, Zhong-Min Wang, Henry Jacob Bonilla, Willard M. Freeman, Osvaldo Delbono
Summary: Maintaining Hand2 expression in SNs from middle-aged to very old mice attenuates decreases in muscle mass and force by preserving sympathetic and motor innervation, improving muscle contraction, preventing inflammation, and increasing muscle autophagy.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Article
Cell Biology
Jakob Proemer, Cinzia Barresi, Ruth Herbst
Summary: Muscle-specific kinase (MuSK) is the key regulator of neuromuscular junction development and its activation and signaling are tightly regulated. Recent studies using omics techniques have contributed to a better understanding of MuSK signaling. Impaired MuSK signaling causes muscle weakness, but the underlying pathophysiology is often unclear. This review focuses on recent advances in deciphering MuSK activation and downstream signaling, as well as the role of MuSK in non-muscle tissue.
CELLULAR SIGNALLING
(2023)
Review
Cell Biology
Osvaldo Delbono, Anna Carolina Zaia Rodrigues, Henry Jacob Bonilla, Maria Laura Messi
Summary: Examining the neural factors involved in the decline of neuromuscular function with aging is crucial to understanding sarcopenia. The sympathetic nervous system may affect muscle composition and function by regulating skeletal muscle motor innervation. Additionally, the role of central neurons may contribute to the coordinated response of the neuromuscular system to physiological and pathological challenges across ages.
AGEING RESEARCH REVIEWS
(2021)
Article
Geriatrics & Gerontology
Jedd Pratt, Giuseppe De Vito, Marco Narici, Ricardo Segurado, Ludmilla Pessanha, Jackie Dolan, Judith Conroy, Colin Boreham
Summary: This study confirms the relevance of plasma CAF as a biomarker for sarcopenia, showing significant associations with muscle mass and elevated levels in sarcopenic individuals. Gender differences were observed, with males showing elevated CAF levels in low grip strength or low ALM, while only low ALM in females had significantly raised CAF concentrations compared to healthy controls.
JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES
(2021)
Article
Physiology
Tatjana Straka, Charlotte Schroder, Andreas Roos, Laxmikanth Kollipara, Albert Sickmann, Marion Patrick Ivey Williams, Mathias Hafner, Muzamil Majid Khan, Rudiger Rudolf
Summary: Recent studies have shown that neuromuscular junctions are co-innervated by sympathetic neurons, which are crucial for maintaining the morphology and function of neuromuscular junctions. Research using various approaches has revealed that sympathetic innervation plays a significant role in muscle and neuromuscular junction homeostasis, leading to changes such as reduced fiber diameters and enhanced acetylcholine receptor turnover. These findings suggest an upregulation of endocytic and autophagic trafficking at the whole muscle level and at the neuromuscular junction.
FRONTIERS IN PHYSIOLOGY
(2021)
Article
Engineering, Biomedical
Agnes Badu-Mensah, Xiufang Guo, Siddharth Nimbalkar, Yunqing Cai, James J. Hickman
Summary: Evidence suggests that pathological defects in ALS human skeletal muscle (hSKM) contribute significantly to neuromuscular junction (NMJ) dysfunction independently of motoneurons. Disease phenotypes are more severe in ALS hSKM compared to healthy hSKM, indicating that therapeutically targeting ALS hSKM may be as critical, if not more critical, than targeting motoneurons in alleviating disease phenotypes and slowing disease progression.
Article
Biochemistry & Molecular Biology
Paola Lorenzon, Sandra Furlan, Barbara Ravara, Alessandra Bosutti, Gabriele Massaria, Annalisa Bernareggi, Marina Sciancalepore, Gabor Trautmann, Katharina Block, Dieter Blottner, Paul F. Worley, Sandra Zampieri, Michele Salanova, Pompeo Volpe
Summary: The study demonstrates that Homer 2 plays a crucial role in skeletal muscle adaptation and plasticity, with specific effects on muscle fiber cross-sectional area and gene expression. Further investigation is needed to determine the impact of Homer 2 deficiency on neuromuscular junction remodeling.
Article
Biochemistry & Molecular Biology
Cristina Pinto, Viviana Perez, Jessica Mella, Miguel Albistur, Teresa Caprile, Francisca C. Bronfman, Juan Pablo Henriquez
Summary: The study demonstrates that Wnt3 is transported and secreted by motor neurons to induce postsynaptic differentiation in nascent NMJs. Overexpression of Wnt3 in NSC-34 cells effectively induces acetylcholine receptor clustering on co-cultured myotubes.
Article
Biochemistry & Molecular Biology
Borja Sanz, Ane Albillos Sanchez, Bonnie Tangey, Kerry Gilmore, Zhilian Yue, Xiao Liu, Gordon Wallace
Summary: Marine collagen, as an alternative source, shows potential in tissue engineering. The study revealed that marine collagen could serve as a low-cost, customizable, scalable, and quick-to-print platform for drug screening and researching neuromuscular junction physiology and pathogenesis.
Article
Cell Biology
Domiziana Costamagna, Valerie Casters, Marc Beltra, Maurilio Sampaolesi, Anja Van Campenhout, Els Ortibus, Kaat Desloovere, Robin Duelen
Summary: This study generated in vitro human neuromuscular junctions (NMJs) using a microfluidic strategy from patient-specific induced pluripotent stem cell (hiPSC) lines to model disease-relevant neuropathologic processes in hereditary spastic paraplegia (HSP). The unique strength of this NMJ model is its ability to generate lower motor neurons (MNs) and myotubes from autologous hiPSC origin while maintaining the genetic background of HSP patient donors. The study found that HSP-derived lines exhibited axonal swellings, reduced levels of SPASTIN protein, and impaired NMJ profiles, offering unique tools to study the pathologic mechanisms of HSP.
Article
Biochemistry & Molecular Biology
Ifigeneia Giakoumaki, Natalie Pollock, Turki Aljuaid, Anthony J. Sannicandro, Moussira Alameddine, Euan Owen, Ioanna Myrtziou, Susan E. Ozanne, Ioannis Kanakis, Katarzyna Goljanek-Whysall, Aphrodite Vasilaki
Summary: Sarcopenia is characterized by a decrease in muscle fibers and weakening of remaining fibers, resulting in reduced muscle mass and function. This study found that low-protein diet during pregnancy and early postnatal low-protein intake in mice can impact skeletal muscle physiology and favor bone integrity in adulthood.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Lisa A. Walter, Lauren P. Blake, Yann S. Gallot, Charles J. Arends, Randall S. Sozio, Stephen M. Onifer, Kyle R. Bohnert
Summary: This study investigates the molecular mechanisms controlling skeletal muscle atrophy following denervation. It shows that transection of the sciatic nerve leads to significant skeletal muscle atrophy in rats, while having little effect on the neuromuscular junction. The study also reveals the activation of the unfolded protein response in denervated skeletal muscle, with ATF4 and ATF6 elevated in the cytoplasm and XBP1 elevated in the nuclei. These findings suggest a potential role of the unfolded protein response and XBP1 in maintaining skeletal muscle and the neuromuscular junction after peripheral nerve injury.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Cell Biology
Madoka Ikemoto-Uezumi, Heying Zhou, Tamaki Kurosawa, Yuki Yoshimoto, Masashi Toyoda, Nobuo Kanazawa, Tatsu Nakazawa, Mitsuhiro Morita, Kunihiro Tsuchida, Akiyoshi Uezumi
Summary: The study reveals an association between increased MFG-E8 levels in skeletal muscle and sarcopenia, suggesting that MFG-E8 may contribute to the degeneration of neuromuscular junctions. Targeting MFG-E8 could be a promising therapeutic approach to prevent sarcopenia.
Article
Geriatrics & Gerontology
Zhong-Min Wang, Anna Carolina Zaia Rodrigues, Maria Laura Messi, Osvaldo Delbono
JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES
(2020)
Article
Geriatrics & Gerontology
Henry J. Bonilla, Maria L. Messi, Khalima A. Sadieva, Craig A. Hamilton, Aron S. Buchman, Osvaldo Delbono
Article
Geriatrics & Gerontology
Anna Carolina Zaia Rodrigues, Zhong-Min Wang, Maria Laura Messi, Henry Jacob Bonilla, Liang Liu, Willard M. Freeman, Osvaldo Delbono
Summary: The study found that inducing the expression of Hand2 in sympathetic neurons in old mice can increase neuron size and number, improve muscle weight and force, enhance muscle innervation, promote muscle transmission, prevent inflammation, and maintain muscle protein synthesis.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Review
Cell Biology
Osvaldo Delbono, Anna Carolina Zaia Rodrigues, Henry Jacob Bonilla, Maria Laura Messi
Summary: Examining the neural factors involved in the decline of neuromuscular function with aging is crucial to understanding sarcopenia. The sympathetic nervous system may affect muscle composition and function by regulating skeletal muscle motor innervation. Additionally, the role of central neurons may contribute to the coordinated response of the neuromuscular system to physiological and pathological challenges across ages.
AGEING RESEARCH REVIEWS
(2021)
Article
Geriatrics & Gerontology
Anna Carolina Zaia Rodrigues, Maria Laura Messi, Zhong-Min Wang, Henry Jacob Bonilla, Willard M. Freeman, Osvaldo Delbono
Summary: Maintaining Hand2 expression in SNs from middle-aged to very old mice attenuates decreases in muscle mass and force by preserving sympathetic and motor innervation, improving muscle contraction, preventing inflammation, and increasing muscle autophagy.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2021)
Review
Physiology
Osvaldo Delbono, Zhong-Min Wang, Maria Laura Messi
Summary: Brainstem noradrenergic neuron clusters play a crucial role in regulating cognition and skeletal muscle function. They are highly heterogeneous and project to different areas in the brain and spinal cord. Understanding the vulnerability of these neurons to protein deposition and aging can help detect neurodegeneration early on and predict disease progression.