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Research Digest
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| Mar. 02 |
Oxidative Stress, Copper Might Be Wrong Suspects in ALS
Subramaniam JR et al. Mutant SOD1 causes motor neuron disease independent of copper chaperone--mediated copper loading. Nat Neurosci. 2002 Mar 11 [PubMed abstract] |
| Mar. 02 |
Unlocking the Mysteries of Guamanian ALS [ALS Newsletter] Galasko D et al. Clinical features and changing patterns of neurodegenerative disorders on Guam, 1997-2000. Neurology. 2002 Jan 8;58(1):90-7. [PubMed abstract] Poorkaj P et al. TAU as a susceptibility gene for amyotropic lateral sclerosis-parkinsonism dementia complex of Guam. Arch Neurol. 2001 Nov;58(11):1871-8. [PubMed abstract] |
| Mar. 02 |
Study of ALS Rat Supports Glutamate Toxicity Idea [ALS Newsletter] Howland DS et al. Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A. 2002 Feb 5;99(3):1604-9. [PubMed abstract] Nagai M et al. Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. J Neurosci. 2001 Dec 1;21(23):9246-54. [PubMed abstract] |
| Mar. 02 |
Chimera: monster or savior? [MDA Technical Summary] Mack TGA et al. Wallerian degeneration of injured axons and synapses is delayed by a Ube4b/Nmnat chimeric gene. Nat Neurosci. 2001 Dec;4(12):1199-206. [PubMed abstract] Wang MS et al. The WldS protein protects against axonal degeneration: a model of gene therapy for peripheral neuropathy. Ann Neurol. 2001 Dec;50(6):773-9. [PubMed abstract] |
| Mar. 02 |
Second ALS Gene Discovered [ALS Newsletter] Yang Y et al. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet. 2001 Oct;29(2):160-5. [PubMed abstract] Hadano, S et al. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet. 2001 Oct;29(2):166-73. [PubMed abstract] |
| Mar. 02 |
HIV Link Could Lead to Anti-Viral Therapy for ALS [ALS Newsletter] MacGowan DJL et al. An ALS-like syndrome with new HIV infection and complete response to antiretroviral therapy. Neurology. 2001 Sep 25;57(6):1094-7. [PubMed abstract] Moulignier A et al. Reversible ALS-like disorder in HIV infection. Neurology. 2001 Sep 25;57(6):995-1001. [PubMed abstract] |
| Nov. 01 |
Vitamin E Might Help a Little
Desnuelle C et al. A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001 Mar;2(1):9-18. [PubMed abstract] [ALS Newsletter] |
| Nov. 01 |
New gene implicated in familial ALS
Oosthuyse B et al. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration. Nat Genet. 2001 Jun;28(2):131-8. [PubMed abstract] [ALS Newsletter] |
| Nov. 01 |
What causes ALS? It's more than motor neurons...
Pramatarova A et al. Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment. J Neurosci. 2001 May 15;21(10):3369-74. [PubMed abstract] [ALS Newsletter] |
| July 01 |
ALS-virus connection still controversial
[MDA Technical Summary] Giraud P et al. Detection of enteroviral sequences from frozen spinal cord samples of Japanese ALS patients. Neurology 2001 Jun 26;56(12):1777-8. [PubMed abstract] Walker MP et al. Absence of echovirus sequences in brain and spinal cord of amyotrophic lateral sclerosis patients. Ann Neurol 2001 Feb;49(2):249-53. [PubMed abstract] |
| July 01 |
No link between ALS and glutamate transporter RNA variants [MDA Technical Summary] Honig LS et al. Glutamate transporter EAAT2 splice variants occur not only in ALS, but also in AD and controls. Neurology 2000 Oct 24;55(8):1082-8. [PubMed abstract] Flowers JM et al. Intron 7 retention and exon 9 skipping EAAT2 mRNA variants are not associated with amyotrophic lateral sclerosis. Ann Neurol. 2001 May;49(5):643-9. [PubMed abstract] |
| July 01 |
Anti-arthritic to be tested against ALS
[ALS Newsletter] Przedborski S et al. Increased expression of the pro-inflammatory enzyme cyclooxygenase-2 in amyotrophic lateral sclerosis. Ann Neurol. 2001 Feb;49(2):176-85. [PubMed abstract] Drachman DB & Rothstein JD. Inhibition of cyclooxygenase-2 protects motor neurons in an organotypic model of amyotrophic lateral sclerosis. Ann Neurol. 2000 Nov;48(5):792-5. [PubMed abstract] |
| July 01 |
A growing connection between ALS and SMA
[ALS Newsletter] [ALS Newsletter] Veldink JH et al. Homozygous deletion of the survival motor neuron 2 gene is a prognostic factor in sporadic ALS. Neurology. 2001 Mar 27;56(6):749-52. [PubMed abstract] Kunst CB et al. Genetic mapping of a mouse modifier gene that can prevent ALS onset. Genomics. 2000 Dec 1;70(2):181-9. [PubMed abstract] |
| Nov. 00 |
Theory suggests sudden death, not slow decline, for nerve cells Clarke G et al. (2000): A one-hit model of cell death in inherited neuronal degenerations. Nature, 406:195-199. [PubMed abstract] [ALS Newsletter] |
| Nov. 00 |
Receptor Subunit Alterations May Render Motor Neurons Hypersensitive to Glutamate in ALS Takuma H, et al. (1999): Reduction of GluR2 editing, a molecular change that increases calcium influx through AMPA receptors, selective in the spinal ventral gray of patients with amyotrophic lateral sclerosis. Annals of Neurology, 46(6):806-815. [PubMed abstract] [MDA Technical Summary] |
| Nov. 00 |
bFGF Not Effective Against ALS in Mice Upton-Rice MN, et al. (1999): Basic Fibroblast Growth Factor does not prolong survival in a transgenic model of familial amyotrophic lateral sclerosis. Annals of Neurology, 46(6):934. [MDA Technical Summary] |
| Nov. 00 |
Evidence Mounts for mitochondrial dysfunction in ALS Borthwick GM, et al. (1999): Mitochondrial Enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death. Annals of Neurology, 46(5):787-790. [PubMed abstract] [MDA Technical Summary] |
| Nov. 00 |
Correlation of Viral Particals and ALS Discovered Berger MM et al. (2000): Detection and cellular localization of enterovirus RNA sequences in spinal cord of patients with ALS. Neurology, 54(1):20-5. [PubMed abstract] [Quest] [FAQ] |
| Nov. 00 |
A Zinc-link in ALS? Estevez AG et al. (1999): Induction of nitric oxide dependent apoptosis in motor neurons by zinc deficient superoxide dismutase. Science, 286(5449):2498-500. [PubMed abstract] [Quest] |
| Dec. 99 |
Piece of AIDS Virus May Prove Useful to Get Therapeutic Proteins Through Blood-Brain Barrier Schwarze SR, et al. (1999): In vivo protein transduction: Delivery of a biologically active protein into the mouse. Science, 285:1560-1572.b [PubMed abstract] [ALS Newsletter] |
| Dec. 99 |
No Signs of Programmed Cell Death in Mice With ALS, One Group Reports Migheli A, et al. (1999): Lack of apoptosis in mice with ALS. Nature Medicine, 5(9):966-967. short report--no abstract available [MDA Technical Summary] |
| Dec. 99 |
Isolated Upper-Limb Weakness--"Man-in-a-Barrel" Syndrome--May Progress Only Slowly Katz JS, et al. (1999): Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder. Neurology, 53:1071-1076. [PubMed abstract] |
| Dec. 99 |
Blocking Active Glutamate Release May Not be Enough to Beat ALS Jabaudon D, et al. (1999): Inhibition of uptake unmasks rapid extracellular turnover of glutamate of nonvesicular origin. Proceedings of the National Academy of Sciences, USA, 96:8733-8738. [PubMed abstract] [MDA Technical Summary] |
| Dec. 99 |
Magnetic Resonance Imaging Technique Can Detect Damage to Spinal Cord Tracts in ALS Ellis CM, et al. (1999): Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology, 53:1051-1058. [PubMed abstract] |
| Dec. 99 |
Gene Therapy Vector Distributes Well in Primate Brains Driesse MJ, et al. (1999): Distribution of recombinant adenovirus in the cerebrospinal fluid of nonhuman primates. Human Gene Therapy, 10(14):2347-2354. [PubMed abstract] |
| Dec. 99 |
Nutrition Impacts Survival in ALS; PEG May Help Desport JC, et al. (1999): Nutritional status is a prognostic factor for survival in ALS patients. Neurology, 53(5): 1059-1063. [PubMed abstract] [MDA Technical Summary] AND Chio A, et al. (1999): Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS, Neurology, 53 (5):1123-1125. [PubMed abstract] [Research Digest?] |
| Dec. 99 |
Researchers Create Hybrid Chemical That Simulataneously Blocks Free Radicals and Potentially Destructive Enzyme Chabrier P-E, et al. (1999): BN 80933, a dual inhibitor of neuronal nitric oxide synthase and lipid peroxidation: A promising neuroprotective strategy. Proceedings of the National Academy of Sciences, 96:10824-10829. [PubMed abstract] |
| Dec. 99 |
PEDF May Prove Good Candidate to Preserve Neurons in ALS Bilak MM, et al. (1999): Pigment epithelium-derived factor (PEDF) protects motor neurons from chronic glutamate-mediated neurodegeneration. Journal of Neuropathology and Experimental Neurology, 58(7):719-28. (MDA-funded) [PubMed abstract] [MDA Technical Summary] [ALS Newsletter] |
| Dec. 99 |
Early Evaluation of Two Proteins That are Related to GDNF: One Shows Promise Against ALS Bilak MM, et al. (1999): Neuroprotective utility and neurotrophic action of neurturin in postnatal motor neurons: comparison with GDNF and persephin. Molecular and Cellular Neuroscience, 13(5):326-36. (MDA-funded.) [PubMed abstract] |
| Sept. 99 |
Magnetic Stimulation Technique Found Effective in Detecting Brain Involvement in ALS Triggs, WJ et al. (1999): Transcranial magnetic stimulation identifies upper motor neuron involvement in motor neuron disease. Neurology, 53 (3):605-611. [PubMed abstract] |
| Sept. 99 |
Slight Decrease in SOD1 Activity Correlated With Slowly Progressive ALS Penco S. et al. (1999): A SOD1 gene mutation in a patient with slowly progressing familial ALS. Neurology, 53(2):404-406. [PubMed abstract] |
| Sept. 99 |
Gene Therapy Breaks Through the Blood-brain Barrier Mojaheri, MH et al. (1999): Intramuscular grafts of myoblasts genetically modified to secrete glial cell line-derived neurotrophic factor prevent motor neuron loss and disease progression in a mouse model of familial amyotrophic lateral sclerosis. Human Gene Therapy, 10:1853-1866. [PubMed abstract] [MDA Technical Summary] |
| Sept. 99 |
Review of Neurotrophic Factors in Neuromuscular Disease (Part 2) Mitsumoto, H and Tsuzaka, K (1999); Neurotrophic factors and neuromuscular disease: II. GDNF, other neurotrophic factors and future directions. Muscle and Nerve22(8):1000-1021. Review. [PubMed abstract] |
| Sept. 99 |
Review of Neurotrophic Factors in Neuromuscular Disease (Part 1) Mitsumoto, H and Tsuzaka, K (1999): Neurotrophic factors and neuromuscular disease: I. General comments, the neurotrophin family and neuropoietic cytokines. Muscle and Nerve, 22(8): 983-999. Review. [PubMed abstract] |
| Sept. 99 |
Backward Falls in ALS Suggest Degeneration in Regions of the Brain That Control Posture Desai, J and Swash, M. (1999): Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls and retropulsion. Journal of Neurosurgery and Psychiatry, 67(2): 214-216. [PubMed abstract] |
| Sept. 99 |
New Brain Cells in All the Right Places Booma DY, et al. (1999): "Global" Stem cell replacement is feasible via neural stem cell transplantation: Evidence from the dysmyelinated shiverer mouse brain. Proceedings of the National Academy of Sciences, USA, 96:7029-7034. [PubMed abstract] [MDA Technical Summary] |
| Sept. 99 |
Study Finds Initial Treatment Preferences Of ALS Patients Consistent With Actual Choices At Later Stages Albert SM et al. (1999): A prospective study of preferences and actual treatment choices in ALS. Neurology, 53(2):278-283. [PubMed abstract] |
| Aug. 99 |
Enzyme Blocker Not Effective Against ALS in Mouse Model Upton-Rice, MN et al. (1999): Administration of nitric oxide synthase inhibitors doesn't alter disease course of amyotrophic lateral sclerosis SOD1 mutant transgenic mice. Annals of Neurology, 45(3):413-414. [PubMed abstract] |
| Aug. 99 |
SOD1 Mutations and Glutamate Transport: Putting the Pieces Together. Trotti, D et al. (May, 1999): SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutatmate transporter. Nature Neuroscience, 2(5):427-433. (MDA funded) [PubMed abstract] [MDA Technical Summary] |
| Aug. 99 |
Copper Chaperone Protein Found in Right Places to Play Role in ALS Rothstein, JD et al. (1999): The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brain. Journal of Neurochemistry, 72:422-429. (MDA funded) [PubMed abstract] |
| Aug. 99 |
Vitamin C, in Combination With Brain/Liver Disease Drug, Delays ALS Onset in Mouse Model Nagano, S et al. (1999): Benefit of a combined treatment with trientine and ascorbate in familial amyotrophic lateral sclerosis model mice. Neuroscience Letters, 265:159-162. [PubMed abstract] |
| Aug. 99 |
Electromyography Results May Suggest That Longer Nerves More Vulnerable In ALS Cappellari, A et al. (1999): A tentative interpretation of electromyographic regional differences in bulbar- and limb-onset ALS. Neurology, 52:644-646. [PubMed abstract] |
| Aug. 99 |
Chaperone Proteins Can Block SOD1 Toxicity in Cultured Cells Bruening, W et al. (1999): Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis. Journal of Neurochemistry, 72:693-699. (MDA funded) [PubMed abstract] |
| Aug. 99 |
Low-Dose Subcutaneous BDNF Not Found Effective Against ALS in Phase III Trial BDNF Study Group (Phase III) (1999): A controlled trial of recombinant methionyl human BDNF in ALS. Neurology, 52:1427-1433. [PubMed abstract] [MDA Technical Summary] [ALS Newsletter] |
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