ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

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Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17

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Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

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Control of microglial neurotoxicity by the fractalkine receptor

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β-Lactam antibiotics offer neuroprotection by increasing glutamate transporter expression

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Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis

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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

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Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man

Fischer, L. R., Culver, D. G., Tennant, P., Davis, A. A., Wang, M., Castellano-Sanchez, A., Khan, J., Polak, M. A., & Glass, J. D. (2004) · Experimental Neurology · doi:10.1016/j.expneurol.2003.10.004 · Cited by 1,357

ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions

Bruijn, L. I., Becher, M. W., Lee, M. K., Anderson, K. L., Jenkins, N. A., Copeland, N. G., Sisodia, S. S., Rothstein, J. D., Borchelt, D. R., Price, D. L., et al. (1997) · Neuron · doi:10.1016/s0896-6273(00)80272-x · Cited by 1,341

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

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Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

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Decreased Glutamate Transport by the Brain and Spinal Cord in Amyotrophic Lateral Sclerosis

Rothstein, J. D., Martin, L. J., & Kuncl, R. W. (1992) · New England Journal of Medicine · doi:10.1056/nejm199205283262204 · Cited by 1,177

Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

Clement, A. M., Nguyen, M. D., Roberts, E. A., Garcia, M. L., Boillée, S., Rule, M., McMahon, A. P., Doucette, W., Siwek, D., Ferrante, R. J., et al. (2003) · Science · doi:10.1126/science.1086071 · Cited by 1,100

Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice

Zhu, S., Stavrovskaya, I. G., Drozda, M., Kim, B. Y. S., Ona, V., Li, M., Sarang, S., Liu, A. S., Hartley, D. M., Wu, D. C., et al. (2002) · Nature · doi:10.1038/417074a · Cited by 1,067

A tetracycline derivative, minocycline, reduces inflammation and protects against focal cerebral ischemia with a wide therapeutic window

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Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration

Oosthuyse, B., Moons, L., Storkebaum, E., Beck, H., Nuyens, D., Brusselmans, K., Dorpe, J. V., Hellings, P., Gorselink, M., Heymans, S., et al. (2001) · Nature Genetics · doi:10.1038/88842 · Cited by 1,007

Mutant dynactin in motor neuron disease

Puls, I., Jonnakuty, C., LaMonte, B. H., Holzbaur, E. L. F., Tokito, M., Mann, E., Floeter, M. K., Bidus, K., Drayna, D., Oh, S. J., et al. (2003) · Nature Genetics · doi:10.1038/ng1123 · Cited by 960

Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria.

Mootha, V. K., Bunkenborg, J., Olsen, J. V., Hjerrild, M., Wisniewski, J. R., Stahl, E., Bolouri, M. S., Ray, H. N., Sihag, S., Kamal, M., et al. (2003) · Cell · doi:10.1016/s0092-8674(03)00926-7 · Cited by 889

A Mutation in the Vesicle-Trafficking Protein VAPB Causes Late-Onset Spinal Muscular Atrophy and Amyotrophic Lateral Sclerosis

Nishimura, A. L., Mitne-Neto, M., Silva, H. C. A., Richieri-Costa, A., Middleton, S., Cascio, D., Kok, F., Oliveira, J. R. M., Gillingwater, T., Webb, J., et al. (2004) · The American Journal of Human Genetics · doi:10.1086/425287 · Cited by 880

Retrograde Viral Delivery of IGF-1 Prolongs Survival in a Mouse ALS Model

Kaspar, B. K., Lladó, J., Sherkat, N., Rothstein, J. D., & Gage, F. H. (2003) · Science · doi:10.1126/science.1086137 · Cited by 872

VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death

Lambrechts, D., Storkebaum, E., Morimoto, M., Del-Favero, J., Desmet, F., Marklund, S. L., Wyns, S., Thijs, V., Andersson, J., van Marion, I., et al. (2003) · Nature Genetics · doi:10.1038/ng1211 · Cited by 860

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)

Howland, D. S., Liu, J., She, Y., Goad, B., Maragakis, N. J., Kim, B., Erickson, J., Kulik, J., DeVito, L., Psaltis, G., et al. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.032539299 · Cited by 841

DNA/RNA Helicase Gene Mutations in a Form of Juvenile Amyotrophic Lateral Sclerosis (ALS4)

Chen, Y.-Z., Bennett, C. L., Huynh, H. M., Blair, I. P., Puls, I., Irobi, J., Dierick, I., Abel, A., Kennerson, M. L., Rabin, B. A., et al. (2004) · The American Journal of Human Genetics · doi:10.1086/421054 · Cited by 808

Charcot-Marie-Tooth Disease Type 2A Caused by Mutation in a Microtubule Motor KIF1Bβ

Zhao, C., Takita, J., Tanaka, Y., Setou, M., Nakagawa, T., Takeda, S., Yang, H. W., Terada, S., Nakata, T., Takei, Y., et al. (2001) · Cell · doi:10.1016/s0092-8674(01)00363-4 · Cited by 767

Microglia Promote the Death of Developing Purkinje Cells

Marı́n-Teva, J. L., Dusart, I., Colin, C., Gervais, A., van Rooijen, N., & Mallat, M. (2004) · Neuron · doi:10.1016/s0896-6273(04)00069-8 · Cited by 757

The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

Yang, Y., Hentati, A., Deng, H.-X., Dabbagh, O., Sasaki, T., Hirano, M., Hung, W.-Y., Ouahchi, K., Yan, J., Azim, A. C., et al. (2001) · Nature Genetics · doi:10.1038/ng1001-160 · Cited by 743

Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study

Turner, M. R., Cagnin, A., Turkheimer, F. E., Miller, C. C. J., Shaw, C. E., Brooks, D. J., Leigh, P. N., & Banati, R. B. (2004) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.12.012 · Cited by 737

Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

Beers, D. R., Henkel, J. S., Xiao, Q., Zhao, W., Wang, J., Yen, A. A., Siklos, L., McKercher, S. R., & Appel, S. H. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0607423103 · Cited by 733

Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1

Kong, J., & Xu, Z. (1998) · The Journal of Neuroscience · doi:10.1523/jneurosci.18-09-03241.1998 · Cited by 723

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis.

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Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model

Li, M., Ona, V. O., Guégan, C., Chen, M., Jackson-Lewis, V., Andrews, L. J., Olszewski, A. J., Stieg, P. E., Lee, J.-P., Przedborski, S., et al. (2000) · Science · doi:10.1126/science.288.5464.335 · Cited by 716

The pharmacology and mechanism of action of riluzole

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Early and Selective Loss of Neuromuscular Synapse Subtypes with Low Sprouting Competence in Motoneuron Diseases

Frey, D., Schneider, C., Xu, L., Borg, J., Spooren, W., & Caroni, P. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-07-02534.2000 · Cited by 687

Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis

Klivenyi, P., Ferrante, R. J., Matthews, R. T., Bogdanov, M. B., Klein, A. M., Andreassen, O. A., Mueller, G., Wermer, M., Kaddurah-Daouk, R., & Beal, M. F. (1999) · Nature Medicine · doi:10.1038/6568 · Cited by 682

A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2

Hadano, S., Hand, C. K., Osuga, H., Yanagisawa, Y., Otomo, A., Devon, R. S., Miyamoto, N., Showguchi-Miyata, J., Okada, Y., Singaraja, R., et al. (2001) · Nature Genetics · doi:10.1038/ng1001-166 · Cited by 678

ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis

Greenway, M. J., Andersen, P. M., Russ, C., Ennis, S., Cashman, S., Donaghy, C., Patterson, V., Swingler, R., Kieran, D., Prehn, J., et al. (2006) · Nature Genetics · doi:10.1038/ng1742 · Cited by 667

Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

Pun, S., Santos, A. F., Saxena, S., Xu, L., & Caroni, P. (2006) · Nature Neuroscience · doi:10.1038/nn1653 · Cited by 658

Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons

Williamson, T. L., & Cleveland, D. W. (1999) · Nature Neuroscience · doi:10.1038/4553 · Cited by 637

Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis

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Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice

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Wallerian degeneration of injured axons and synapses is delayed by a Ube4b/Nmnat chimeric gene

Mack, T. G. A., Reiner, M., Beirowski, B., Mi, W., Emanuelli, M., Wagner, D., Thomson, D., Gillingwater, T., Court, F., Conforti, L., et al. (2001) · Nature Neuroscience · doi:10.1038/nn770 · Cited by 611

Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis

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VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model

Azzouz, M., Ralph, G. S., Storkebaum, E., Walmsley, L. E., Mitrophanous, K. A., Kingsman, S. M., Carmeliet, P., & Mazarakis, N. D. (2004) · Nature · doi:10.1038/nature02544 · Cited by 590

Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy

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Presence of dendritic cells, MCP‐1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord tissue

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Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis

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Microtubule-Based Transport Systems in Neurons: The Roles of Kinesins and Dyneins

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Immunologic reactions in amyotrophic lateral sclerosis brain and spinal cord tissue.

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Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease

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A Kinesin Heavy Chain (KIF5A) Mutation in Hereditary Spastic Paraplegia (SPG10)

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RNA editing and death of motor neurons

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Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice

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COX-2, CB2 and P2X7-immunoreactivities are increased in activated microglial cells/macrophages of multiple sclerosis and amyotrophic lateral sclerosis spinal cord

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Antisense oligonucleotide therapy for neurodegenerative disease

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Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria

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Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria

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Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model

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Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS

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Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS

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Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment

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Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

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Global Survey of Organ and Organelle Protein Expression in Mouse: Combined Proteomic and Transcriptomic Profiling

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Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17

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Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis

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Cytoplasmic dynein binds dynactin through a direct interaction between the intermediate chains and p150Glued.

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Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS

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Mutations in the Small GTP-ase Late Endosomal Protein RAB7 Cause Charcot-Marie-Tooth Type 2B Neuropathy

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A controlled trial of recombinant methionyl human BDNF in ALS

The BDNF Study Group (Phase III) (1999) · Neurology · doi:10.1212/wnl.52.7.1427 · Cited by 451

Motoneuron Death Triggered by a Specific Pathway Downstream of Fas

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Histological Evidence of Protein Aggregation in Mutant SOD1 Transgenic Mice and in Amyotrophic Lateral Sclerosis Neural Tissues

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Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

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Accumulation of SOD1 Mutants in Postnatal Motoneurons Does Not Cause Motoneuron Pathology or Motoneuron Disease

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Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

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Minocycline Slows Disease Progression in a Mouse Model of Amyotrophic Lateral Sclerosis

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A double‐blind placebo‐controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis

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Complete Dissociation of Motor Neuron Death from Motor Dysfunction by Bax Deletion in a Mouse Model of ALS

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Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.

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Restricted Expression of G86R Cu/Zn Superoxide Dismutase in Astrocytes Results in Astrocytosis But Does Not Cause Motoneuron Degeneration

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Oxygen‐induced maturation of SOD1: a key role for disulfide formation by the copper chaperone CCS

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Fine Structural Observations of Neurofilamentous Changes in Amyotrophic Lateral Sclerosis

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