ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease

Akaaboune, 2000, Developmental regulation of amyloid precursor protein at the neuromuscular junction in mouse skeletal muscle, Mol. Cell. Neurosci, 15, 355, 10.1006/mcne.2000.0834

Akbik, 2013, Anatomical plasticity of adult brain is titrated by Nogo Receptor 1, Neuron, 77, 859, 10.1016/j.neuron.2012.12.027

Alabed, 2007, Identification of CRMP4 as a convergent regulator of axon outgrowth inhibition, J. Neurosci, 27, 1702, 10.1523/JNEUROSCI.5055-06.2007

Alami, 2014, Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations, Neuron, 81, 536, 10.1016/j.neuron.2013.12.018

Alfahad, 2013, Retroviruses and amyotrophic lateral sclerosis, Antiviral Res, 99, 180, 10.1016/j.antiviral.2013.05.006

Arendt, 2009, Synaptic degeneration in Alzheimer's disease, Acta Neuropathol. (Berl.), 118, 167, 10.1007/s00401-009-0536-x

Arimura, 2009, CRMP-2 directly binds to cytoplasmic dynein and interferes with its activity, J. Neurochem, 111, 380, 10.1111/j.1471-4159.2009.06317.x

Arnold, 2013, ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43, Proc. Natl. Acad. Sci. U. S. A, 110, E736, 10.1073/pnas.1222809110

Artuso, 2013, Mitochondrial genome aberrations in skeletal muscle of patients with motor neuron disease, Amyotroph. Lateral Scler. Frontotemporal Degener, 14, 261, 10.3109/21678421.2012.735239

Atkin, 2005, Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis, Neuromuscul. Disord, 15, 377, 10.1016/j.nmd.2005.02.005

Auld, 2003, Glial cells and neurotransmission: an inclusive view of synaptic function, Neuron, 40, 389, 10.1016/S0896-6273(03)00607-X

Azzouz, 2004, VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model, Nature, 429, 413, 10.1038/nature02544

Belzil, 2013, RNA-mediated toxicity in neurodegenerative disease, Mol. Cell. Neurosci, 56, 406, 10.1016/j.mcn.2012.12.006

Blasco, 2013, A rare motor neuron deleterious missense mutation in the DPYSL3 (CRMP4) gene is associated with ALS, Hum. Mutat, 34, 953, 10.1002/humu.22329

Blijham, 2007, Early diagnosis of ALS: the search for signs of denervation in clinically normal muscles, J. Neurol. Sci, 263, 154, 10.1016/j.jns.2007.07.008

Blokhuis, 2013, Protein aggregation in amyotrophic lateral sclerosis, Acta Neuropathol. (Berl.), 125, 777, 10.1007/s00401-013-1125-6

Boillée, 2006, ALS: a disease of motor neurons and their nonneuronal neighbors, Neuron, 52, 39, 10.1016/j.neuron.2006.09.018

Bongioanni, 2004, Ciliary neurotrophic factor (CNTF) for amyotrophic lateral sclerosis/motor neuron disease, Cochrane Database Syst. Rev, CD004302, 10.1002/14651858.CD004302.pub2

Bourke, 2006, Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial, Lancet Neurol, 5, 140, 10.1016/S1474-4422(05)70326-4

Bradley, 2013, Is exposure to cyanobacteria an environmental risk factor for amyotrophic lateral sclerosis and other neurodegenerative diseases?, Amyotroph. Lateral Scler. Frontotemporal. Degener, 14, 325, 10.3109/21678421.2012.750364

Bros-Facer, 2014, Treatment with an antibody directed against Nogo-A delays disease progression in the SOD1G93A mouse model of Amyotrophic lateral sclerosis, Hum. Mol. Genet, 23, 4187, 10.1093/hmg/ddu136

Brot, 2010, CRMP5 interacts with tubulin to inhibit neurite outgrowth, thereby modulating the function of CRMP2, J. Neurosci, 30, 10639, 10.1523/JNEUROSCI.0059-10.2010

Bryson, 2012, Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis, Hum. Mol. Genet, 21, 3871, 10.1093/hmg/dds215

Burke, 1973, Physiological types and histochemical profiles in motor units of the cat gastrocnemius, J. Physiol, 234, 723, 10.1113/jphysiol.1973.sp010369

Caillol, 2012, Motor endplate disease affects neuromuscular junction maturation, Eur. J. Neurosci, 36, 2400, 10.1111/j.1460-9568.2012.08164.x

Camby, 2006, Galectin-1: a small protein with major functions, Glycobiology 16, 137R, 10.1093/glycob/cwl025

Casas, 2013, Early presymptomatic cholinergic dysfunction in a murine model of amyotrophic lateral sclerosis, Brain Behav, 3, 145, 10.1002/brb3.104

Cedarbaum, 1997, Performance of the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) in multicenter clinical trials, J. Neurol. Sci, 152, S1, 10.1016/S0022-510X(97)00237-2

Chang-Hong, 2005, Neuroprotective effect of oxidized galectin-1 in a transgenic mouse model of amyotrophic lateral sclerosis, Exp. Neurol, 194, 203, 10.1016/j.expneurol.2005.02.011

Chiò, 2005, Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players, Brain, 128, 472, 10.1093/brain/awh373

Chiò, 2009, ALS in Italian professional soccer players: the risk is still present and could be soccer-specific, Amyotroph. Lateral Scler, 10, 205, 10.1080/17482960902721634

Choi, 2013, APP interacts with LRP4 and agrin to coordinate the development of the neuromuscular junction in mice, Elife, 2, e00220, 10.7554/eLife.00220

Covault, 1985, Neural cell adhesion molecule (N-CAM) accumulates in denervated and paralyzed skeletal muscles, Proc. Natl. Acad. Sci. U.S.A, 82, 4544, 10.1073/pnas.82.13.4544

Cox, 2009, Cyanobacteria and BMAA exposure from desert dust: a possible link to sporadic ALS among Gulf War veterans, Amyotroph. Lateral Scler, 10, 109, 10.3109/17482960903286066

Dauer, 2003, Parkinson's disease: mechanisms and models, Neuron, 39, 889, 10.1016/S0896-6273(03)00568-3

De Carvalho, 2008, Electrodiagnostic criteria for diagnosis of ALS, Clin. Neurophysiol, 119, 497, 10.1016/j.clinph.2007.09.143

de Carvalho, 2013, Fasciculation potentials and earliest changes in motor unit physiology in ALS, J. Neurol. Neurosurg. Psychiatry, 84, 963, 10.1136/jnnp-2012-304545

De Munck, 2013, β-N-methylamino-l-alanine causes neurological and pathological phenotypes mimicking Amyotrophic Lateral Sclerosis (ALS): the first step towards an experimental model for sporadic ALS, Environ. Toxicol. Pharmacol, 36, 243, 10.1016/j.etap.2013.04.007

Deng, 2014, The role of FUS gene variants in neurodegenerative diseases, Nat. Rev. Neurol, 10, 337, 10.1038/nrneurol.2014.78

Deng, 2006, Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria, Proc. Natl. Acad. Sci, 103, 7142, 10.1073/pnas.0602046103

Dengler, 1990, Amyotrophic lateral sclerosis: macro-EMG and twitch forces of single motor units, Muscle Nerve, 13, 545, 10.1002/mus.880130612

Deo, 2004, Structural bases for CRMP function in plexin-dependent semaphorin3A signaling, EMBO J, 23, 9, 10.1038/sj.emboj.7600021

De Winter, 2006, The expression of the chemorepellent Semaphorin 3A is selectively induced in terminal Schwann cells of a subset of neuromuscular synapses that display limited anatomical plasticity and enhanced vulnerability in motor neuron disease, Mol. Cell. Neurosci, 32, 102, 10.1016/j.mcn.2006.03.002

Do, 2011, Growth factor regulation of neural chemorepellent Sema3A expression in satellite cell cultures, Am. J. Physiol. Cell Physiol, 301, C1270, 10.1152/ajpcell.00257.2011

Dobrowolny, 2005, Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model, J. Cell Biol, 168, 193, 10.1083/jcb.200407021

Droppelmann, 2014, RNA metabolism in ALS: when normal processes become pathological, Amyotroph. Lateral Scler. Frontotemporal Degener, 10.3109/21678421.2014.881377

Duplan, 2010, Collapsin response mediator protein 4a (CRMP4a) is upregulated in motoneurons of mutant SOD1 mice and can trigger motoneuron axonal degeneration and cell death, J. Neurosci, 30, 785, 10.1523/JNEUROSCI.5411-09.2010

Dupuis, 2014, Mitochondrial quality control in neurodegenerative diseases, Biochimie, 100, 177, 10.1016/j.biochi.2013.07.033

Dupuis, 2002, Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis, Neurobiol. Dis, 10, 358, 10.1006/nbdi.2002.0522

Dupuis, 2009, Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons, PLoS ONE, 4, e5390, 10.1371/journal.pone.0005390

Dupuis, 2009, Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models, Curr. Opin. Pharmacol, 9, 341, 10.1016/j.coph.2009.03.007

Fallini, 2012, The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth, Hum. Mol. Genet, 21, 3703, 10.1093/hmg/dds205

Fanara, 2007, Stabilization of hyperdynamic microtubules is neuroprotective in amyotrophic lateral sclerosis, J. Biol. Chem, 282, 23465, 10.1074/jbc.M703434200

Ferraiuolo, 2007, Microarray analysis of the cellular pathways involved in the adaptation to and progression of motor neuron injury in the SOD1 G93A mouse model of familial ALS, J. Neurosci, 27, 9201, 10.1523/JNEUROSCI.1470-07.2007

Filosto, 2011, The role of mitochondria in neurodegenerative diseases, J. Neurol, 258, 1763, 10.1007/s00415-011-6104-z

Fischer, 2004, Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man, Exp. Neurol, 185, 232, 10.1016/j.expneurol.2003.10.004

Fischer, 2011, SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse, Brain, 134, 196, 10.1093/brain/awq314

Fischer, 2012, Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy, Exp. Neurol, 233, 163, 10.1016/j.expneurol.2011.09.020

Fogh, 2014, A genome-wide association meta-analysis identifies a novel locus at 17q11.2 associated with sporadic amyotrophic lateral sclerosis, Hum. Mol. Genet, 23, 2220, 10.1093/hmg/ddt587

Forbes, 2004, Frequency, timing and outcome of gastrostomy tubes for amyotrophic lateral sclerosis/motor neurone disease–a record linkage study from the scottish motor neurone disease register, J. Neurol, 251, 813, 10.1007/s00415-004-0429-9

Frey, 2000, Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases, J. Neurosci, 20, 2534, 10.1523/JNEUROSCI.20-07-02534.2000

Fukata, 2002, CRMP-2 binds to tubulin heterodimers to promote microtubule assembly, Nat. Cell Biol, 4, 583, 10.1038/ncb825

Garcia, 2010, Localization of brain-derived neurotrophic factor, neurotrophin-4, tropomyosin-related kinase b receptor, and p75 NTR receptor by high-resolution immunohistochemistry on the adult mouse neuromuscular junction, J. Peripher. Nerv. Syst, 15, 40, 10.1111/j.1529-8027.2010.00250.x

Garratt, 2000, Neuregulin, a factor with many functions in the life of a schwann cell, Bioessays, 22, 987, 10.1002/1521-1878(200011)22:11%3C987::AID-BIES5%3E3.3.CO;2-X

Gendron, 2014, Mechanisms of toxicity in C9FTLD/ALS, Acta Neuropathol. (Berl.), 127, 359, 10.1007/s00401-013-1237-z

Goldman, 1988, Acetylcholine receptor α-, β-, γ-, and δ-subunit mRNA levels are regulated by muscle activity, Neuron, 1, 329, 10.1016/0896-6273(88)90081-5

Gonzalez de Aguilar, 2008, Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model, Physiol. Genomics, 32, 207, 10.1152/physiolgenomics.00017.2007

Goodison, 1999, CD44 cell adhesion molecules, Mol. Pathol, 52, 189, 10.1136/mp.52.4.189

Gordon, 2004, Adaptive and maladaptive motor axonal sprouting in aging and motoneuron disease, Neurol. Res, 26, 174, 10.1179/016164104225013806

Gordon, 2009, Early detection of denervated muscle fibers in hindlimb muscles after sciatic nerve transection in wild type mice and in the G93A mouse model of amyotrophic lateral sclerosis, Neurol. Res, 31, 28, 10.1179/174313208X332977

Gordon, 2010, Functional over-load saves motor units in the SOD1-G93A transgenic mouse model of amyotrophic lateral sclerosis, Neurobiol. Dis, 37, 412, 10.1016/j.nbd.2009.10.021

Gorlewicz, 2009, CD44 is expressed in non-myelinating Schwann cells of the adult rat, and may play a role in neurodegeneration-induced glial plasticity at the neuromuscular junction, Neurobiol. Dis, 34, 245, 10.1016/j.nbd.2009.01.011

Goshima, 1995, Collapsin-induced growth cone collapse mediated by an intracellular protein related to UNC-33, Nature, 376, 509, 10.1038/376509a0

Gould, 2006, Complete dissociation of motor neuron death from motor dysfunction by bax deletion in a mouse model of ALS, J. Neurosci, 26, 8774, 10.1523/JNEUROSCI.2315-06.2006

Gu, 2000, Evidence that collapsin response mediator protein-2 is involved in the dynamics of microtubules, J. Biol. Chem, 275, 17917, 10.1074/jbc.C000179200

Gurney, 1994, Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation, Science, 264, 1772, 10.1126/science.8209258

Hammad, 2007, Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS, Neurology, 69, 2236, 10.1212/01.wnl.0000286948.99150.16

Hegedus, 2007, Time course of preferential motor unit loss in the SOD1G93A mouse model of amyotrophic lateral sclerosis, Neurobiol. Dis, 28, 154, 10.1016/j.nbd.2007.07.003

Hegedus, 2008, Preferential motor unit loss in the SOD1 G93A transgenic mouse model of amyotrophic lateral sclerosis, J. Physiol, 586, 3337, 10.1113/jphysiol.2007.149286

Henriques, 2010, Neurotrophic growth factors for the treatment of amyotrophic lateral sclerosis: where do we stand?, Front. Neurosci, 4, 10.3389/fnins.2010.00032

Hensley, 2010, Proteomic identification of binding partners for the brain metabolite lanthionine ketimine (LK) and documentation of LK effects on microglia and motoneuron cell cultures, J. Neurosci, 30, 2979, 10.1523/JNEUROSCI.5247-09.2010

Hensley, 2011, Collapsin response mediator protein-2: an emerging pathologic feature and therapeutic target for neurodisease indications, Mol. Neurobiol, 43, 180, 10.1007/s12035-011-8166-4

Hess, 2007, Localization of TrkC to Schwann cells and effects of neurotrophin-3 signaling at neuromuscular synapses, J. Comp. Neurol, 501, 465, 10.1002/cne.21163

Hida, 2012, GSK3β/axin-1/β-catenin complex is involved in semaphorin3A signaling, J. Neurosci, 32, 11905, 10.1523/JNEUROSCI.6139-11.2012

Horie, 2004, Galectin-1 plays essential roles in adult mammalian nervous tissues. Roles of oxidized galectin-1, Glycoconj. J, 19, 479, 10.1023/B:GLYC.0000014077.84016.52

Hsieh, 2008, Galectin-1, a novel ligand of neuropilin-1, activates VEGFR-2 signaling and modulates the migration of vascular endothelial cells, Oncogene, 27, 3746, 10.1038/sj.onc.1211029

Hu, 2006, Bace1 modulates myelination in the central and peripheral nervous system, Nat. Neurosci, 9, 1520, 10.1038/nn1797

Igoudjil, 2011, In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space, J. Neurosci, 31, 15826, 10.1523/JNEUROSCI.1965-11.2011

Iwase, 2005, Glial cell line-derived neurotrophic factor-induced signaling in Schwann cells, J. Neurochem, 94, 1488, 10.1111/j.1471-4159.2005.03290.x

Jessen, 2002, Signals that determine Schwann cell identity, J. Anat, 200, 367, 10.1046/j.1469-7580.2002.00046.x

Jokic, 2006, The neurite outgrowth inhibitor Nogo-A promotes denervation in an amyotrophic lateral sclerosis model, EMBO Rep, 7, 1162, 10.1038/sj.embor.7400826

Jokic, 2005, Nogo expression in muscle correlates with amyotrophic lateral sclerosis severity, Ann. Neurol, 57, 553, 10.1002/ana.20420

Kanai, 2006, Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage, Brain J. Neurol, 129, 953, 10.1093/brain/awl024

Kaspar, 2003, Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model, Science, 301, 839, 10.1126/science.1086137

Kato, 2001, Galectin-1 is a component of neurofilamentous lesions in sporadic and familial amyotrophic lateral sclerosis, Biochem. Biophys. Res. Commun, 282, 166, 10.1006/bbrc.2001.4556

Kato, 2005, Galectin-1 as a potential therapeutic agent for amyotrophic lateral sclerosis, Curr. Drug Targets, 6, 407, 10.2174/1389450054021846

Keifer, 2014, Gene and protein therapies utilizing VEGF for ALS, Pharmacol. Ther, 141, 261, 10.1016/j.pharmthera.2013.10.009

Keller, 2009, Live imaging of amyotrophic lateral sclerosis pathogenesis: disease onset is characterized by marked induction of GFAP in Schwann cells, Glia, 57, 1130, 10.1002/glia.20836

Khanna, 2012, Opening Pandora's jar: a primer on the putative roles of CRMP2 in a panoply of neurodegenerative, sensory and motor neuron, and central disorders, Future Neurol, 7, 749, 10.2217/fnl.12.68

Killian, 1994, Decremental motor responses to repetitive nerve stimulation in ALS, Muscle Nerve, 17, 747, 10.1002/mus.880170708

Klein, 2009, Bidirectional modulation of synaptic functions by Eph/ephrin signaling, Nat. Neurosci, 12, 15, 10.1038/nn.2231

Koistinen, 2006, Elevated levels of amyloid precursor protein in muscle of patients with amyotrophic lateral sclerosis and a mouse model of the disease, Muscle Nerve, 34, 444, 10.1002/mus.20612

Kostic, 1997, Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis, Science, 277, 559, 10.1126/science.277.5325.559

Krakora, 2012, Neuromuscular junction protection for the potential treatment of amyotrophic lateral sclerosis, Neurol. Res. Int, 2012, 1, 10.1155/2012/379657

Kye, 2014, The role of miRNA in motor neuron disease, Front. Cell. Neurosci, 8, 10.3389/fncel.2014.00015

Laaksovirta, 2010, Chromosome 9p21 in amyotrophic lateral sclerosis in Finland: a genome-wide association study, Lancet Neurol, 9, 978, 10.1016/S1474-4422(10)70184-8

Lagier-Tourenne, 2012, Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs, Nat. Neurosci, 15, 1488, 10.1038/nn.3230

Lalonde, 2003, Neurobehavioral characteristics of mice with modified intermediate filament genes, Rev. Neurosci, 14, 369, 10.1515/REVNEURO.2003.14.4.369

Lambrechts, 2003, VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death, Nat. Genet, 34, 383, 10.1038/ng1211

LaMonte, 2002, Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration, Neuron, 34, 715, 10.1016/S0896-6273(02)00696-7

Lee, 2012, Oligodendroglia metabolically support axons and contribute to neurodegeneration, Nature, 487, 443, 10.1038/nature11314

Lehman, 2012, Neurodegenerative causes of death among retired National Football League players, Neurology, 79, 1970, 10.1212/WNL.0b013e31826daf50

Lemmens, 2013, Modifying expression of EphA4 and its downstream targets improves functional recovery after stroke, Hum. Mol. Genet, 22, 2214, 10.1093/hmg/ddt073

Lesnick, 2008, Beyond parkinson disease: amyotrophic lateral sclerosis and the axon guidance pathway, PLoS ONE, 3, e1449, 10.1371/journal.pone.0001449

Li, 2007, Muscle-derived but not centrally derived transgene GDNF is neuroprotective in G93A-SOD1 mouse model of ALS, Exp. Neurol, 203, 457, 10.1016/j.expneurol.2006.08.028

Libeu, 2012, Altering APP Proteolysis: increasing sAPPalpha production by targeting dimerization of the APP ectodomain, PLoS ONE, 7, e40027, 10.1371/journal.pone.0040027

Ling, 2013, Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis, Neuron, 79, 416, 10.1016/j.neuron.2013.07.033

Liu, 2004, Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria, Neuron, 43, 5, 10.1016/j.neuron.2004.06.016

Liu, 2014, C9ORF72 mutations in neurodegenerative diseases, Mol. Neurobiol, 49, 386, 10.1007/s12035-013-8528-1

Llado, 2013, Cellular and molecular mechanisms involved in the neuroprotective effects of VEGF on motoneurons, Front. Cell. Neurosci, 7, 10.3389/fncel.2013.00181

Lobsiger, 2009, Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice, Proc. Natl. Acad. Sci. U.S.A, 106, 4465, 10.1073/pnas.0813339106

Ludolph, 2012, Amyotrophic lateral sclerosis, Curr. Opin. Neurol, 25, 530, 10.1097/WCO.0b013e328356d328

Luo, 2013, Defective mitochondrial dynamics is an early event in skeletal muscle of an amyotrophic lateral sclerosis mouse model, PLoS ONE, 8, e82112, 10.1371/journal.pone.0082112

Machamer, 2014, The ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction, Hum. Mol. Genet, 23, 3810, 10.1093/hmg/ddu094

Magdesian, 2011, Secreted human amyloid precursor protein binds semaphorin 3a and prevents semaphorin-induced growth cone collapse, PLoS ONE, 6, e22857, 10.1371/journal.pone.0022857

Magill, 2010, The differential effects of pathway- versus target-derived glial cell line-derived neurotrophic factor on peripheral nerve regeneration, J. Neurosurg, 113, 102, 10.3171/2009.10.JNS091092

Mancuso, 2014, Pre-symptomatic electrophysiological tests predict clinical onset and survival in SOD1(G93A) ALS mice, Muscle Nerve, 10.1002/mus.24237

Mancuso, 2011, Electrophysiological analysis of a murine model of motoneuron disease, Clin. Neurophysiol. Off. J. Int. Fed. Clin. Neurophysiol, 122, 1660, 10.1016/j.clinph.2011.01.045

Manzano, 2013, Altered in vitro proliferation of mouse SOD1-G93A skeletal muscle satellite cells, Neurodegener. Dis, 11, 153, 10.1159/000338061

Manzano, 2011, Altered expression of myogenic regulatory factors in the mouse model of amyotrophic lateral sclerosis, Neurodegener. Dis, 8, 386, 10.1159/000324159

Matsumoto, 2012, CD44 expression in astrocytes and microglia is associated with ALS progression in a mouse model, Neurosci. Lett, 520, 115, 10.1016/j.neulet.2012.05.048

Mikl, 2010, RNA localization in neurite morphogenesis and synaptic regulation: current evidence and novel approaches, J. Comp. Physiol. A Neuroethol. Sens. Neural. Behav. Physiol, 196, 321, 10.1007/s00359-010-0520-x

Miller, 2012, Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND), Cochrane Database Syst. Rev, 3, CD001447

Moloney, 2012, AAV-mediated delivery of neuropilin-1 receptor bodies to the neuromuscular junction of G93A-hSOD1 ALS mice, Soc. Neurosci. Program No, 15607I3

Muresan, 2014, Functional interaction between amyloid-β precursor protein and peripherin neurofilaments: a shared pathway leading to Alzheimer's disease and amyotrophic lateral sclerosis?, Neurodegener. Dis, 13, 122, 10.1159/000354238

Nakata, 2006, Distal excitability changes in motor axons in amyotrophic lateral sclerosis, Clin. Neurophysiol. Off. J. Int. Fed. Clin. Neurophysiol, 117, 1444, 10.1016/j.clinph.2006.04.005

Nalivaeva, 2013, The amyloid precursor protein: a biochemical enigma in brain development, function and disease, FEBS Lett, 587, 2046, 10.1016/j.febslet.2013.05.010

Narendra, 2012, Neurodegeneration: trouble in the cell's powerhouse, Nature, 483, 418, 10.1038/nature10952

Nguyen, 1998, Hyperinnervation of neuromuscular junctions caused by GDNF overexpression in muscle, Science, 279, 1725, 10.1126/science.279.5357.1725

Nikolaev, 2009, APP binds DR6 to trigger axon pruning and neuron death via distinct caspases, Nature, 457, 981, 10.1038/nature07767

Noto, 2011, Distal motor axonal dysfunction in amyotrophic lateral sclerosis, J. Neurol. Sci, 302, 58, 10.1016/j.jns.2010.11.025

O'Brien, 2011, Amyloid precursor protein processing and Alzheimer's disease, Annu. Rev. Neurosci, 34, 185, 10.1146/annurev-neuro-061010-113613

O'Hanlon, 2003, Calpain inhibitors protect against axonal degeneration in a model of anti−ganglioside antibody−mediated motor nerve terminal injury, Brain, 126, 2497, 10.1093/brain/awg254

Obál, 2006, Axotomy induces contrasting changes in calcium and calcium-binding proteins in oculomotor and hypoglossal nuclei of Balb/c mice, J. Comp. Neurol, 499, 17, 10.1002/cne.21041

Octave, 2013, From synaptic spines to nuclear signaling: nuclear and synaptic actions of the amyloid precursor protein, J. Neurochem, 126, 183, 10.1111/jnc.12239

Okita, 2011, Can Awaji ALS criteria provide earlier diagnosis than the revised El Escorial criteria?, J. Neurol. Sci, 302, 29, 10.1016/j.jns.2010.12.007

Olbrich, 2013, Fast rearrangement of the neuronal growth cone's actin cytoskeleton following VEGF stimulation, Histochem. Cell Biol, 139, 431, 10.1007/s00418-012-1036-y

Pansarasa, 2014, Amyotrophic lateral sclerosis and skeletal muscle: an update, Mol. Neurobiol, 49, 984, 10.1007/s12035-013-8578-4

Parone, 2013, Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis, J. Neurosci. Off. J. Soc. Neurosci, 33, 4657, 10.1523/JNEUROSCI.1119-12.2013

Pawlik, 2007, Proteins that bind to the RERMS region of beta amyloid precursor protein, Biochem. Biophys. Res. Commun, 355, 907, 10.1016/j.bbrc.2007.02.047

Peng, 2011, Neuronal Nogo-A regulates glutamate receptor subunit expression in hippocampal neurons: Nogo-A regulation of glutamate receptors, J. Neurochem, 119, 1183, 10.1111/j.1471-4159.2011.07520.x

Pernet, 2012, The role of Nogo-A in axonal plasticity, regrowth and repair, Cell Tissue Res, 349, 97, 10.1007/s00441-012-1432-6

Phatnani, 2013, Intricate interplay between astrocytes and motor neurons in ALS, Proc. Natl. Acad. Sci. U. S. A, 110, E756, 10.1073/pnas.1222361110

Philips, 2013, Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis, Brain J. Neurol, 136, 471, 10.1093/brain/aws339

Phukan, 2009, The management of amyotrophic lateral sclerosis, J. Neurol, 256, 176, 10.1007/s00415-009-0142-9

Plachta, 2007, Identification of a lectin causing the degeneration of neuronal processes using engineered embryonic stem cells, Nat. Neurosci, 10, 712, 10.1038/nn1897

Polymenidou, 2012, Misregulated RNA processing in amyotrophic lateral sclerosis, Brain Res, 1462, 3, 10.1016/j.brainres.2012.02.059

Pradat, 2011, Abnormalities of satellite cells function in amyotrophic lateral sclerosis, Amyotroph. Lateral Scler. Off. Publ. World Fed. Neurol. Res. Group Mot. Neuron Dis, 12, 264, 10.3109/17482968.2011.566618

Pradat, 2007, Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes, Ann. Neurol, 62, 15, 10.1002/ana.21122

Priller, 2006, Synapse formation and function is modulated by the amyloid precursor protein, J. Neurosci, 26, 7212, 10.1523/JNEUROSCI.1450-06.2006

Puls, 2003, Mutant dynactin in motor neuron disease, Nat. Genet, 33, 455, 10.1038/ng1123

Pun, 2006, Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF, Nat. Neurosci, 9, 408, 10.1038/nn1653

Rabinovich-Toidman, 2012, Inhibition of amyloid precursor protein beta-secretase cleavage site affects survival and motor functions of amyotrophic lateral sclerosis transgenic mice, Neurodegener. Dis, 10, 30, 10.1159/000334774

Rafalowska, 2010, Is the spinal cord motoneuron exclusively a target in ALS? Comparison between astroglial reactivity in a rat model of familial ALS and in human sporadic ALS cases, Neurol. Res, 32, 867, 10.1179/174313209X414542

Rahajeng, 2010, Collapsin response mediator protein-2 (Crmp2) regulates trafficking by linking endocytic regulatory proteins to dynein motors, J. Biol. Chem, 285, 31918, 10.1074/jbc.C110.166066

Raman, 2014, Gene expression signatures in motor neuron disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functions, Neuropathol. Appl. Neurobiol, 10.1111/nan.12147

Redler, 2012, The complex molecular biology of amyotrophic lateral sclerosis (ALS), Prog. Mol. Biol. Transl. Sci, 107, 215, 10.1016/B978-0-12-385883-2.00002-3

Renton, 2014, State of play in amyotrophic lateral sclerosis genetics, Nat. Neurosci, 17, 17, 10.1038/nn.3584

Renton, 2011, A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD, Neuron, 72, 257, 10.1016/j.neuron.2011.09.010

Riethmacher, 1997, Severe neuropathies in mice with targeted mutations in the ErbB3 receptor, Nature, 389, 725, 10.1038/39593

Robberecht, 2013, The changing scene of amyotrophic lateral sclerosis, Nat. Rev. Neurosci, 14, 248, 10.1038/nrn3430

Rocha, 2013, Early changes of neuromuscular transmission in the SOD1(G93A) mice model of ALS start long before motor symptoms onset, PLoS ONE, 8, e73846, 10.1371/journal.pone.0073846

Rolls, 2008, Two faces of chondroitin sulfate proteoglycan in spinal cord repair: a role in microglia/macrophage activation, PLoS Med, 5, e171, 10.1371/journal.pmed.0050171

Rosen, 1993, Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis, Nature, 362, 59, 10.1038/362059a0

Rutishauser, 1985, Influences of the neural cell adhesion molecule on axon growth and guidance, J. Neurosci. Res, 13, 123, 10.1002/jnr.490130109

Sato, 2013, Satellite cells produce neural chemorepellent semaphorin 3A upon muscle injury, Anim. Sci. J, 84, 185, 10.1111/asj.12014

Saxena, 2009, A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice, Nat. Neurosci, 12, 627, 10.1038/nn.2297

Schaefer, 2005, A compensatory subpopulation of motor neurons in a mouse model of amyotrophic lateral sclerosis, J. Comp. Neurol, 490, 209, 10.1002/cne.20620

Schmidt, 2007, The CRMP family of proteins and their role in Sema3A signaling, Adv. Exp. Med. Biol, 600, 1, 10.1007/978-0-387-70956-7_1

Schmidt, 2009, Axon guidance proteins: novel therapeutic targets for ALS?, Prog. Neurobiol, 88, 286, 10.1016/j.pneurobio.2009.05.004

Schmidt, 2011, Neuregulin/ErbB regulate neuromuscular junction development by phosphorylation of α-dystrobrevin, J. Cell Biol, 195, 1171, 10.1083/jcb.201107083

Schmied, 1999, Electromechanical coupling and synchronous firing of single wrist extensor motor units in sporadic amyotrophic lateral sclerosis, Clin. Neurophysiol. Off. J. Int. Fed. Clin. Neurophysiol, 110, 960, 10.1016/S1388-2457(99)00032-2

Seale, 2000, A new look at the origin, function, and “stem-cell” status of muscle satellite cells, Dev. Biol, 218, 115, 10.1006/dbio.1999.9565

Selkoe, 2002, Alzheimer's disease is a synaptic failure, Science, 298, 789, 10.1126/science.1074069

Selvaraj, 2012, Local axonal function of STAT3 rescues axon degeneration in the pmn model of motoneuron disease, J. Cell Biol, 199, 437, 10.1083/jcb.201203109

Shatunov, 2010, Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study, Lancet Neurol, 9, 986, 10.1016/S1474-4422(10)70197-6

Sherman, 2000, Cd44 Enhances Neuregulin Signaling by Schwann Cells, J. Cell Biol, 150, 1071, 10.1083/jcb.150.5.1071

Simon, 2010, Ciliary neurotrophic factor-induced sprouting preserves motor function in a mouse model of mild spinal muscular atrophy, Hum. Mol. Genet, 19, 973, 10.1093/hmg/ddp562

Soler-Martín, 2012, Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy, Neuropathol. Appl. Neurobiol, 38, 61, 10.1111/j.1365-2990.2011.01178.x

Son, 1995, Schwann cell processes guide regeneration of peripheral axons, Neuron, 14, 125, 10.1016/0896-6273(95)90246-5

Son, 1996, Schwann cells induce and guide sprouting and reinnervation of neuromuscular junctions, Trends Neurosci, 19, 280, 10.1016/S0166-2236(96)10032-1

Sondell, 1999, Vascular endothelial growth factor stimulates Schwann cell invasion and neovascularization of acellular nerve grafts, Brain Res, 846, 219, 10.1016/S0006-8993(99)02056-9

Sotelo-Silveira, 2009, Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS, Antioxid. Redox Signal, 11, 1535, 10.1089/ars.2009.2614

Storkebaum, 2005, Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS, Nat. Neurosci, 8, 85, 10.1038/nn1360

Stowell, 2009, Ligand reduces galectin-1 sensitivity to oxidative inactivation by enhancing dimer formation, J. Biol. Chem, 284, 4989, 10.1074/jbc.M808925200

Sugiura, 2011, Neuron-glia interactions: the roles of Schwann cells in neuromuscular synapse formation and function, Biosci. Rep, 31, 295, 10.1042/BSR20100107

Suzuki, 2008, Direct muscle delivery of GDNF with human mesenchymal stem cells improves motor neuron survival and function in a rat model of familial ALS, Mol. Ther. J. Am. Soc. Gene Ther, 16, 2002, 10.1038/mt.2008.197

Tabata, 2008, Chronic exposure to dietary sterol glucosides is neurotoxic to motor neurons and induces an ALS-PDC phenotype, Neuromolecular Med, 10, 24, 10.1007/s12017-007-8020-z

Takahashi, 2013, ERBB4 mutations that disrupt the neuregulin-ErbB4 pathway cause amyotrophic lateral sclerosis Type 19, Am. J. Hum. Genet, 93, 900, 10.1016/j.ajhg.2013.09.008

Takata, 2013, Fasudil, a rho kinase inhibitor, limits motor neuron loss in experimental models of amyotrophic lateral sclerosis, Br. J. Pharmacol, 170, 341, 10.1111/bph.12277

Tam, 2003a, Mechanisms controlling axonal sprouting at the neuromuscular junction, J. Neurocytol, 32, 961, 10.1023/B:NEUR.0000020635.41233.0f

Tam, 2003b, Neuromuscular activity impairs axonal sprouting in partially denervated muscles by inhibiting bridge formation of perisynaptic Schwann cells, J. Neurobiol, 57, 221, 10.1002/neu.10276

Tatsumi, 2009, Possible implication of satellite cells in regenerative motoneuritogenesis: HGF upregulates neural chemorepellent Sema3A during myogenic differentiation, Am. J. Physiol. Cell Physiol, 297, C238, 10.1152/ajpcell.00161.2009

Tjust, 2012, Unaffected motor endplate occupancy in eye muscles of ALS G93A mouse model, Front. Biosci. Sch. Ed, 4, 1547, 10.2741/S351

Todd, 2007, Neurotrophins modulate neuron-glia interactions at a vertebrate synapse, Eur. J. Neurosci, 25, 1287, 10.1111/j.1460-9568.2007.05385.x

Trachtenberg, 1997, Nerve terminal withdrawal from rat neuromuscular junctions induced by Neuregulin and Schwann cells, J. Neurosci, 17, 6243, 10.1523/JNEUROSCI.17-16-06243.1997

Trojsi, 2013, Exposure to environmental toxicants and pathogenesis of amyotrophic lateral sclerosis: state of the art and research perspectives, Int. J. Mol. Sci, 14, 15286, 10.3390/ijms140815286

Turner, 2010, Dismutase-competent SOD1 mutant accumulation in myelinating Schwann cells is not detrimental to normal or transgenic ALS model mice, Hum. Mol. Genet, 19, 815, 10.1093/hmg/ddp550

Turner, 2003, Roles of amyloid precursor protein and its fragments in regulating neural activity, plasticity and memory, Prog. Neurobiol, 70, 1, 10.1016/S0301-0082(03)00089-3

Uchida, 2005, Semaphorin3A signalling is mediated via sequential Cdk5 and GSK3beta phosphorylation of CRMP2: implication of common phosphorylating mechanism underlying axon guidance and Alzheimer's disease, Genes Cells Devoted Mol. Cell. Mech, 10, 165, 10.1111/j.1365-2443.2005.00827.x

Valdez, 2012, Shared resistance to aging and ALS in neuromuscular junctions of specific muscles, PLoS ONE, 7, e34640, 10.1371/journal.pone.0034640

Valori, 2014, The multifaceted role of glial cells in amyotrophic lateral sclerosis, Cell. Mol. Life Sci, 71, 287, 10.1007/s00018-013-1429-7

Vande Velde, 2008, Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria, Proc. Natl. Acad. Sci. U.S.A, 105, 4022, 10.1073/pnas.0712209105

Van Hoecke, 2012, EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans, Nat. Med, 18, 1418, 10.1038/nm.2901

Van Zundert, 2008, Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis, J. Neurosci, 28, 10864, 10.1523/JNEUROSCI.1340-08.2008

Venkova, 2014, Semaphorin 3A signaling through Neuropilin-1 is an early trigger for distal axonopathy in the SOD1G93A mouse model of amyotrophic lateral sclerosis, J. Neuropathol. Exp. Neurol, 73, 702, 10.1097/NEN.0000000000000086

Vucic, 2006, Axonal excitability properties in amyotrophic lateral sclerosis, Clin. Neurophysiol. Off. J. Int. Fed. Clin. Neurophysiol, 117, 1458, 10.1016/j.clinph.2006.04.016

Weisskopf, 2009, Prospective study of chemical exposures and amyotrophic lateral sclerosis, J. Neurol. Neurosurg. Psychiatry, 80, 558, 10.1136/jnnp.2008.156976

Willem, 2006, Control of peripheral nerve myelination by the ß-Secretase BACE1, Science, 314, 664, 10.1126/science.1132341

Williams, 2009, MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice, Science, 326, 1549, 10.1126/science.1181046

Williamson, 2011, CRMP2 hyperphosphorylation is characteristic of Alzheimer's disease and not a feature common to other neurodegenerative diseases, J. Alzheimers Dis, 27, 615, 10.3233/JAD-2011-110617

Williamson, 1998, Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant, Proc. Natl. Acad. Sci. U.S.A, 95, 9631, 10.1073/pnas.95.16.9631

Williamson, 1999, Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons, Nat. Neurosci, 2, 50, 10.1038/4553

Wolpowitz, 2000, Cysteine-rich domain isoforms of the neuregulin-1 gene are required for maintenance of peripheral synapses, Neuron, 25, 79, 10.1016/S0896-6273(00)80873-9

Wu, 2012, Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis, Nature, 488, 499, 10.1038/nature11280

Yamanaka, 2008a, Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice, Proc. Natl. Acad. Sci. U.S.A, 105, 7594, 10.1073/pnas.0802556105

Yamanaka, 2008b, Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis, Nat. Neurosci, 11, 251, 10.1038/nn2047

Yamashita, 2012, Collapsin response mediator proteins regulate neuronal development and plasticity by switching their phosphorylation status, Mol. Neurobiol, 45, 234, 10.1007/s12035-012-8242-4

Yang, 2008, Galectins: structure, function and therapeutic potential, Expert Rev. Mol. Med, 10, e17, 10.1017/S1462399408000719

Yoshimura, 2005, GSK-3β regulates phosphorylation of CRMP-2 and neuronal polarity, Cell, 120, 137, 10.1016/j.cell.2004.11.012

Zheng, 2007, VEGF reduces astrogliosis and preserves neuromuscular junctions in ALS transgenic mice, Biochem. Biophys. Res. Commun, 363, 989, 10.1016/j.bbrc.2007.09.088

Zhou, 2010, Galectin-3 is a candidate biomarker for ALS: discovery by a proteomics approach, J. Proteome Res, 9, 5133, 10.1021/pr100409r

Zhou, 2012, Why are the neurodegenerative disease-related pathways overrepresented in primary HIV-infected peripheral blood mononuclear cells: a genome-wide perspective, Virol. J, 9, 308, 10.1186/1743-422X-9-308

Zwick, 2001, Overexpression of GDNF induces and maintains hyperinnervation of muscle fibers and multiple end-plate formation, Exp. Neurol, 171, 342, 10.1006/exnr.2001.7753