Genetic evidence that relative synaptic efficacy biases the outcome of synaptic competition.
AuthorsBuffelli M, Burgess RW, Feng G, Lobe CG, Lichtman JW, Sanes JR
JournalNature
PubMed ID12879071
'Synaptic activity drives synaptic rearrangement in the vertebrate nervous system; indeed, this appears to be a main way in which experience shapes neural connectivity. One rearrangement that occurs in many parts of the nervous system during early postnatal life is a competitive process called ''synapse elimination''. At the neuromuscular junction, ... More
Impaired neuromuscular transmission and skeletal muscle fiber necrosis in mice lacking Na/Ca exchanger 3.
AuthorsSokolow S, Manto M, Gailly P, Molgó J, Vandebrouck C, Vanderwinden JM, Herchuelz A, Schurmans S
JournalJ Clin Invest
PubMed ID14722618
'We produced and analyzed mice deficient for Na/Ca exchanger 3 (NCX3), a protein that mediates cellular Ca(2+) efflux (forward mode) or Ca(2+) influx (reverse mode) and thus controls intracellular Ca(2+) concentration. NCX3-deficient mice (Ncx3(-/-)) present a skeletal muscle fiber necrosis and a defective neuromuscular transmission, reflecting the absence of NCX3 ... More
The use of amphipols as universal molecular adapters to immobilize membrane proteins onto solid supports.
'Because of the importance of their physiological functions, cell membranes represent critical targets in biological research. Membrane proteins, which make up approximately 1/3 of the proteome, interact with a wide range of small ligands and macromolecular partners as well as with foreign molecules such as synthetic drugs, antibodies, toxins, or ... More
Axonal neuregulin 1 is a rate limiting but not essential factor for nerve remyelination.
AuthorsFricker FR, Antunes-Martins A, Galino J, Paramsothy R, La Russa F, Perkins J, Goldberg R, Brelstaff J, Zhu N, McMahon SB, Orengo C, Garratt AN, Birchmeier C, Bennett DL,
Journal
PubMed ID23801741
Neuregulin 1 acts as an axonal signal that regulates multiple aspects of Schwann cell development including the survival and migration of Schwann cell precursors, the ensheathment of axons and subsequent elaboration of the myelin sheath. To examine the role of this factor in remyelination and repair following nerve injury, we ... More
Axonally derived neuregulin-1 is required for remyelination and regeneration after nerve injury in adulthood.
AuthorsFricker FR, Lago N, Balarajah S, Tsantoulas C, Tanna S, Zhu N, Fageiry SK, Jenkins M, Garratt AN, Birchmeier C, Bennett DL,
JournalJ Neurosci
PubMed ID21368034
Neuregulin-1 (NRG1) plays a crucial role in axoglial signaling during the development of the peripheral nervous system, but its importance in adulthood after peripheral nerve injury remains unclear. We used single-neuron labeling with inducible Cre-mediated knock-out animals, which enabled visualization of a subset of adult myelinated sensory and motoneurons neurons ... More
Kir2.6 regulates the surface expression of Kir2.x inward rectifier potassium channels.
AuthorsDassau L, Conti LR, Radeke CM, Ptácek LJ, Vandenberg CA,
JournalJ Biol Chem
PubMed ID21209095
Precise trafficking, localization, and activity of inward rectifier potassium Kir2 channels are important for shaping the electrical response of skeletal muscle. However, how coordinated trafficking occurs to target sites remains unclear. Kir2 channels are tetrameric assemblies of Kir2.x subunits. By immunocytochemistry we show that endogenous Kir2.1 and Kir2.2 are localized ... More
Hyperactive intracellular calcium signaling associated with localized mitochondrial defects in skeletal muscle of an animal model of amyotrophic lateral sclerosis.
AuthorsZhou J, Yi J, Fu R, Liu E, Siddique T, Ríos E, Deng HX,
JournalJ Biol Chem
PubMed ID19889637
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder characterized by degeneration of motor neurons and atrophy of skeletal muscle. Mutations in the superoxide dismutase (SOD1) gene are linked to 20% cases of inherited ALS. Mitochondrial dysfunction has been implicated in the pathogenic process, but how it contributes to muscle ... More
Induction of neuregulin signaling in mouse schwann cells in vivo mimics responses to denervation.
AuthorsHayworth CR, Moody SE, Chodosh LA, Krieg P, Rimer M, Thompson WJ
JournalJ Neurosci
PubMed ID16793894
Neuregulins play crucial roles in early development of Schwann cells (SCs), but their roles in the activities of SCs during denervation and reinnervation of muscle are less clear. In the present study, the Tet-On system has been used in transgenic mice to enable inducible expression of a mutant, constitutively active ... More
Quantification of human neuromuscular function through optogenetics.
Authors
JournalTheranostics
PubMed ID30867827
Novel neuromuscular junction model in 2D and 3D myotubes co-cultured with induced pluripotent stem cell-derived motor neurons.
AuthorsYoshioka K, Ito A, Kawabe Y, Kamihira M
JournalJ Biosci Bioeng
PubMed ID31678066
'Motor neurons differentiated from induced pluripotent stem (iPS) cells have attracted attention for use in the construction of drug screening systems for neuronal diseases, such as amyotrophic lateral sclerosis. However, conventional drug screening systems using 2-dimensional (2D) cultures of iPS cell-derived motor neurons often evaluate the cell survival rate, morphological ... More
Evidence of structural and functional plasticity occurring within the intracardiac nervous system of spontaneously hypertensive rats.
AuthorsAshton JL, Argent L, Smith JEG, Jin S, Sands GB, Smaill BH, Montgomery JM
JournalAm J Physiol Heart Circ Physiol
PubMed ID32357112
'Plasticity is a fundamental property of neurons in both the central and peripheral nervous systems, enabling rapid changes in neural network function. The intracardiac nervous system (ICNS) is an extensive network of neurons clustered into ganglionated plexi (GP) on the surface of the heart. GP neurons are the final site ... More
Presynaptic Homeostasis Opposes Disease Progression in Mouse Models of ALS-Like Degeneration: Evidence for Homeostatic Neuroprotection.
AuthorsOrr BO, Hauswirth AG, Celona B, Fetter RD, Zunino G, Kvon EZ, Zhu Y, Pennacchio LA, Black BL, Davis GW
JournalNeuron
PubMed ID32380032
'Progressive synapse loss is an inevitable and insidious part of age-related neurodegenerative disease. Typically, synapse loss precedes symptoms of cognitive and motor decline. This suggests the existence of compensatory mechanisms that can temporarily counteract the effects of ongoing neurodegeneration. Here, we demonstrate that presynaptic homeostatic plasticity (PHP) is induced at ... More
Treatment of a Mouse Model of ALS by In Vivo Base Editing.
Authors
JournalMol Ther
PubMed ID31991108
Synaptogenesis Stimulates a Proteasome-Mediated Ribosome Reduction in Axons.
Authors
JournalCell Rep
PubMed ID31340150
Polyamine regulation of ion channel assembly and implications for nicotinic acetylcholine receptor pharmacology.
Authors
JournalNat Commun
PubMed ID32493979
Knockout of reactive astrocyte activating factors slows disease progression in an ALS mouse model.
Authors
JournalNat Commun
PubMed ID32719333
Human sensorimotor organoids derived from healthy and amyotrophic lateral sclerosis stem cells form neuromuscular junctions.
Authors
JournalNat Commun
PubMed ID34362895
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
Authors
JournalActa Neuropathol
PubMed ID32562018
Progress of endocytic CHRN to autophagic degradation is regulated by RAB5-GTPase and T145 phosphorylation of SH3GLB1 at mouse neuromuscular junctions in vivo.
Authors
JournalAutophagy
PubMed ID27715385
Presynaptic Homeostasis Opposes Disease Progression in Mouse Models of ALS-Like Degeneration: Evidence for Homeostatic Neuroprotection.
Authors
JournalNeuron
PubMed ID32380032
Role of autophagy, SQSTM1, SH3GLB1, and TRIM63 in the turnover of nicotinic acetylcholine receptors.
Authors
JournalAutophagy
PubMed ID24220501
Muscle specific kinase protects dystrophic mdx mouse muscles from eccentric contraction-induced loss of force-producing capacity.
AuthorsTrajanovska S, Ban J, Huang J, Gregorevic P, Morsch M, Allen DG, Phillips WD
JournalJ Physiol
PubMed ID31340406
Adeno-associated viral vector was used to elevate the expression of muscle specific kinase (MuSK) and rapsyn (a cytoplasmic MuSK effector protein) in the tibialis anterior muscle of wild-type and dystrophic (mdx) mice. In mdx mice, enhanced expression of either MuSK or rapsyn ameliorated the acute loss of muscle force associated ... More
Focal and dose-dependent neuroprotection in ALS mice following AAV2-neurturin delivery.
Neurotrophic factors as candidates for ALS therapeutics have previously been studied in the context of attempts to slow disease progression. For a variety of reasons, clinical trials of neurotrophic factors have failed to show efficacy in ALS patients. Previous studies in Parkinson's Disease (PD) models have shown promise with the ... More