Results 101 to 110 of about 73,691 (271)

ErbB2 signaling in Schwann cells is mostly dispensable for maintenance of myelinated peripheral nerves and proliferation of adult Schwann cells after injury [PDF]

, 2006
Neuregulin/erbB signaling is critically required for survival and proliferation of Schwann cells as well as for establishing correct myelin thickness of peripheral nerves during development.
Atanasoski, Suzana, Birchmeier, C., Garratt, Alistair N., Birchmeier, Carmen, Suter, U., Sirkowski, E., Scherer, Steven S., Atanasoski, S., Suter, Ueli, Scherer, S.S., Sirkowski, Erich, Garratt, A.N., S. Atanasoski, Leone, Dino, Leone, D.   +14 more
core   +1 more source

From energy provision to protein synthesis: Tunnelling nanotubes as mediators of intercellular metabolic cooperation in cancer

FEBS Open Bio, EarlyView.
The cytoskeleton‐mediated transport of mitochondria via tunnelling nanotubes restores respiration, increases ATP production, rescues cells from apoptosis, activates the AKT/mTOR signalling pathway, promotes cell migration and invasiveness, contributes to cancer progression and treatment resistance.
Stanislava Martínková, Jan Trnka
wiley   +1 more source

Investigations of Microtubule-associated Protein 2 Gene Expression in Spinal Muscular Atrophy

Journal of Pediatric Research, 2019
Aim:Spinal muscular atrophy (SMA) is a devastating genetic disease in childhood andff is caused by the absence of functional survival motor neuron (SMN) protein, which leads to impairments of the cytoskeleton, especially in neurons.
Gamze Bora, Ceren Sucularlı, Niko Hensel, Peter Claus, Hayat Erdem Yurter   +4 more
doaj   +1 more source

Fluid Biomarkers of Disease Burden and Cognitive Dysfunction in Progressive Supranuclear Palsy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Identifying objective biomarkers for progressive supranuclear palsy (PSP) is crucial to improving diagnosis and establishing clinical trial and treatment endpoints. This study evaluated fluid biomarkers in PSP versus controls and their associations with regional 18F‐PI‐2620 tau‐PET, clinical, and cognitive outcomes.
Roxane Dilcher, Charles B. Malpas, Stuart J. McDonald, William T. O’Brien, Craig Despott, Kelly L. Bertram, Matthew P. Pase, Lukas Frontzkowski, Meng Law, Terence J. O’Brien, Lucy Vivash   +10 more
wiley   +1 more source

Onasemnogene Abeparvovec in Type I Spinal Muscular Atrophy: 24‐Month Follow‐Up From the Italian Registry

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Onasemnogene abeparvovec (OA) is an AAV9‐based gene therapy for spinal muscular atrophy type I (SMA I). Real‐world outcomes show increased response variability compared to clinical trials, and follow‐up data beyond 12–18 months are limited.
Marika Pane, Giorgia Coratti, Chiara Cutrì, Antonio Varone, Riccardo Masson, Adele D'Amico, Valeria Sansone, Sonia Messina, Federica Ricci, Chiara Ticci, Claudio Bruno, Caterina Agosto, Francesca Benedetti, Antonella Pini, Sabrina Siliquini, Massimiliano Filosto, Alberto Zambon, Ilaria Bitetti, Maria Rosaria Manna, Claudia Dosi, Riccardo Zanin, Stefano Parravicini, Roberto De Sanctis, Giulia Stanca, Michela Catteruccia, Michele Tosi, Irene Mizzoni, Emilio Albamonte, Valentina Franchino, Maria Sframeli, Ilaria Cavallina, Elena Procopio, Michele Sacchini, Simone Morando, Noemi Brolatti, Federica Trucco, Gaia Scarpini, Elena Briganti, Beatrice Berti, Concetta Palermo, Daniela Leone, Stefano C. Previtali, Eugenio Mercuri, the ITASMAC working group   +43 more
wiley   +1 more source

Study of survival of motor neuron (SMN) and neuronal apoptosis inhibitory protein (NAIP) gene deletions in SMA patients [PDF]

, 2005
In view of the paucity of deletion studies of survival of motor neuron (SMN) and neuronal apoptosis inhibitor protein (NAIP) genes in Indian SMA patients, this study has been undertaken to determine the status of SMN1, SMN2 and NAIP gene deletions in ...
Phadke, S. R., Misra, U. K., Kalita, J., Mishra, V. N., Mittal, B., Patil, S. J., Kesari, A., Pradhan, S.   +7 more
core   +1 more source

ALDOA Promotes Glycolysis and NLRP3/GSDMD Pyroptosis to Accelerate ALS Progression

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is characterized by progressive motor neuron degeneration. Glycolytic dysregulation is implicated in disease progression, yet the underlying mechanisms remain unclear. This study investigates how Aldolase A (ALDOA) drives ALS progression through glycolysis‐mediated motor neuron pyroptosis.
Kaixin Yan, Yan Jiang, Yuxuan Yong, Tianshuo Zhang, Niannian Zhang, Qianqian Zeng, Xue Gong, Li Meng, Fangfang Bi, Yongmin Liu   +9 more
wiley   +1 more source

Drug Candidate BIO101 for Spinal Muscular Atrophy as Monotherapy or Combined With the Antisense Oligonucleotide ASO‐10‐27

Journal of Cachexia, Sarcopenia and Muscle
Background Spinal muscular atrophy (SMA) is a neuromuscular disease caused by loss of survival of motor neuron (SMN) protein inducing progressive muscle weakness and atrophy due to motor neurons degeneration. Despite benefits of SMN restoration therapies
Cynthia Bézier, Steve Cottin, Parvin Nazari Hashemi, Mirella El Khoury, Zoé Clerc, Christine Balducci, Delphine Sapaly, Laure Weill, René Lafont, Stanislas Veillet, Pierre J. Dilda, Frédéric Charbonnier, Mathilde Latil, Olivier Biondi   +13 more
doaj   +1 more source

Alteration of protein folding and degradation in motor neuron diseases: Implications and protective functions of small heat shock proteins

, 2012
Motor neuron diseases (MNDs) are neurodegenerative disorders that specifically affect the survival and function of upper and/or lower motor neurons.
CARRA, Serena, Angelo Poletti, Poletti, Angelo, Melania Minoia, Crippa, V, Harm H. Kampinga, A. Boncoraglio, Rusmini, P, Elisa Giorgetti, P. Rusmini, Minoia, M, Kampinga, Harm H.; id_orcid, E. Giorgetti, H. H. Kampinga, Giorgetti, E, Minoia, Melania, Alessandra Boncoraglio, Serena Carra, Rusmini, Paola, Boncoraglio, Alessandra, S. Carra, M. Minoia, V. Crippa, Boncoraglio, A, A. Poletti, Kampinga, HH, Giorgetti, Elisa, Paola Rusmini, Poletti, A., Kampinga, Harm H., Crippa, Valeria, Valeria Crippa   +31 more
core   +1 more source

Neuronal deletion of GSK3beta increases microtubule speed in the growth cone and enhances axon regeneration via CRMP-2 and independently of MAP1B and CLASP2 [PDF]

, 2014
BackgroundIn the adult central nervous system, axonal regeneration is abortive. Regulators of microtubule dynamics have emerged as attractive targets to promote axonal growth following injury as microtubule organization is pivotal for growth cone ...
Sílvia Vieira, Wittmann, Torsten, Sousa, Mónica M., Márcia A Liz, Pimentel, Helena I., Vera F Sousa, Telma E Santos, Vieira, Sílvia, Marques, Ana M., Sutherland, Calum; id_orcid, Hayley Pemble, Liz, Márcia A., Calum Sutherland, Mar, Fernando M., Mónica M Sousa, Pemble, Hayley, Sousa, Vera F., Helena I Pimentel, James R Woodgett, Morgado, Marlene M., Torsten Wittmann, Woodgett, James R., Santos, Telma E., Fernando M Mar, Ana M Marques, Marlene M Morgado   +25 more
core   +1 more source