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Previous Volume 340:1970-1980 June 24, 1999 Number 25 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Neurodegenerative disorders, which are chronic and progressive, are characterized by selective and symmetric loss of neurons in motor, sensory, or cognitive systems. Delineation of the patterns of cell loss and the identification of disease-specific cellular markers have aided in nosologic classification: senile plaques, neurofibrillary tangles, neuronal loss, and acetylcholine deficiency define Alzheimer's disease^1,2; Lewy bodies and depletion of dopamine characterize Parkinson's disease³; cellular inclusions and swollen motor axons are found in amyotrophic lateral sclerosis⁴; and -aminobutyric acid–containing neurons of the neostriatum are lost in Huntington's disease.⁵

Mendelian inheritance can be demonstrated in many of these disorders (. . . [Full Text of this Article]

Alzheimer's Disease

Pathogenesis

The Amyloid Hypothesis

Transgenic Mice and Alzheimer's Disease

Role of Tau in the Formation of Neurofibrillary Tangles

Frontotemporal Dementia Associated with Mutations in Tau

Disorders of Trinucleotide Repeats

Huntington's Disease

Functions of the Proteasome

Spinocerebellar Ataxias

Friedreich's Ataxia

Parkinson's Disease

The Role of -Synuclein in Other Neurologic Disorders

Amyotrophic Lateral Sclerosis and Other Motor-Neuron Disorders

Amyotrophic Lateral Sclerosis

Spinal Muscular Atrophy

Spinal and Bulbar Muscular Atrophy

Familial Spastic Paraparesis

Conclusions

Source Information

From Harvard Medical School, Boston.

Address reprint requests to Dr. Martin at Harvard Medical School, 25 Shattuck St., Rm. A111, Boston, MA 02115, or at joseph_martin@hms.harvard.edu.

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• Soto, C., Estrada, L. D. (2008). Protein Misfolding and Neurodegeneration. Arch Neurol 65: 184-189 [Abstract] [Full Text]  
• Shivaprasad, S., Wetzel, R. (2006). Scanning Cysteine Mutagenesis Analysis of Abeta-(1-40) Amyloid Fibrils. J. Biol. Chem. 281: 993-1000 [Abstract] [Full Text]  
• Seki, T., Adachi, N., Ono, Y., Mochizuki, H., Hiramoto, K., Amano, T., Matsubayashi, H., Matsumoto, M., Kawakami, H., Saito, N., Sakai, N. (2005). Mutant Protein Kinase C{gamma} Found in Spinocerebellar Ataxia Type 14 Is Susceptible to Aggregation and Causes Cell Death. J. Biol. Chem. 280: 29096-29106 [Abstract] [Full Text]  
• Wells, R. D., Dere, R., Hebert, M. L., Napierala, M., Son, L. S. (2005). Advances in mechanisms of genetic instability related to hereditary neurological diseases. Nucleic Acids Res 33: 3785-3798 [Abstract] [Full Text]  
• Castro-Fernandez, C., Maya-Nunez, G., Conn, P. M. (2005). Beyond the Signal Sequence: Protein Routing in Health and Disease. Endocr. Rev. 26: 479-503 [Abstract] [Full Text]  
• Salnikova, A. B., Kryndushkin, D. S., Smirnov, V. N., Kushnirov, V. V., Ter-Avanesyan, M. D. (2005). Nonsense Suppression in Yeast Cells Overproducing Sup35 (eRF3) Is Caused by Its Non-heritable Amyloids. J. Biol. Chem. 280: 8808-8812 [Abstract] [Full Text]  
• Jana, A., Pahan, K. (2004). Fibrillar Amyloid-{beta} Peptides Kill Human Primary Neurons via NADPH Oxidase-mediated Activation of Neutral Sphingomyelinase: IMPLICATIONS FOR ALZHEIMER'S DISEASE. J. Biol. Chem. 279: 51451-51459 [Abstract] [Full Text]  
• Mascalchi, M., Lolli, F., Nave, R. D., Tessa, C., Petralli, R., Gavazzi, C., Politi, L. S., Macucci, M., Filippi, M., Piacentini, S. (2004). Huntington Disease: Volumetric, Diffusion-weighted, and Magnetization Transfer MR Imaging of Brain. Radiology 232: 867-873 [Abstract] [Full Text]  
• Guerrini, L., Lolli, F., Ginestroni, A., Belli, G., Nave, R. D., Tessa, C., Foresti, S., Cosottini, M., Piacentini, S., Salvi, F., Plasmati, R., De Grandis, D., Siciliano, G., Filla, A., Mascalchi, M. (2004). Brainstem neurodegeneration correlates with clinical dysfunction in SCA1 but not in SCA2. A quantitative volumetric, diffusion and proton spectroscopy MR study. Brain 127: 1785-1795 [Abstract] [Full Text]  
• Frikke-Schmidt, R., Nordestgaard, B. G., Schnohr, P., Tybjaerg-Hansen, A. (2004). Single nucleotide polymorphism in the low-density lipoprotein receptor is associated with a threefold risk of stroke: A case-control and prospective study. Eur Heart J 25: 943-951 [Abstract] [Full Text]  
• Seth, P., Diaz, F., Tao-Cheng, J.-H., Major, E. O. (2004). JC Virus Induces Nonapoptotic Cell Death of Human Central Nervous System Progenitor Cell-Derived Astrocytes. J. Virol. 78: 4884-4891 [Abstract] [Full Text]  
• Pedersen, C. B., Bross, P., Winter, V. S., Corydon, T. J., Bolund, L., Bartlett, K., Vockley, J., Gregersen, N. (2003). Misfolding, Degradation, and Aggregation of Variant Proteins: THE MOLECULAR PATHOGENESIS OF SHORT CHAIN ACYL-CoA DEHYDROGENASE (SCAD) DEFICIENCY. J. Biol. Chem. 278: 47449-47458 [Abstract] [Full Text]  
• Tedde, A., Nacmias, B., Ciantelli, M., Forleo, P., Cellini, E., Bagnoli, S., Piccini, C., Caffarra, P., Ghidoni, E., Paganini, M., Bracco, L., Sorbi, S. (2003). Identification of New Presenilin Gene Mutations in Early-Onset Familial Alzheimer Disease. Arch Neurol 60: 1541-1544 [Abstract] [Full Text]  
• Martinez, J., Moeller, I., Erdjument-Bromage, H., Tempst, P., Lauring, B. (2003). Parkinson's Disease-associated alpha -Synuclein Is a Calmodulin Substrate. J. Biol. Chem. 278: 17379-17387 [Abstract] [Full Text]  
• Nussbaum, R. L., Ellis, C. E. (2003). Alzheimer's Disease and Parkinson's Disease. NEJM 348: 1356-1364 [Full Text]  
• Friedlander, R. M. (2003). Apoptosis and Caspases in Neurodegenerative Diseases. NEJM 348: 1365-1375 [Full Text]  
• Prasad, K. N., Cole, W. C., Prasad, K. C. (2002). Risk Factors for Alzheimer's Disease: Role of Multiple Antioxidants, Non-Steroidal Anti-inflammatory and Cholinergic Agents Alone or in Combination in Prevention and Treatment. J. Am. Coll. Nutr. 21: 506-522 [Abstract] [Full Text]  
• Gilchrist, D. M. (2002). Medical genetics: 3. An approach to the adult with a genetic disorder. CMAJ 167: 1021-1029 [Abstract] [Full Text]  
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• Sinclair, A. (2002). Genetics 101: detecting mutations in human genes. CMAJ 167: 275-279 [Full Text]  
• Pilch, J., Jamroz, E., Marsza, E. (2002). Topical Review : Friedreich's Ataxia. J Child Neurol 17: 315-319 [Abstract]  
• O'Nuallain, B., Wetzel, R. (2002). Conformational Abs recognizing a generic amyloid fibril epitope. Proc. Natl. Acad. Sci. USA 10.1073/pnas.022662599v1 [Abstract] [Full Text]  
• Uryu, K., Laurer, H., McIntosh, T., Pratico, D., Martinez, D., Leight, S., Lee, V. M.-Y., Trojanowski, J. Q. (2002). Repetitive Mild Brain Trauma Accelerates Abeta Deposition, Lipid Peroxidation, and Cognitive Impairment in a Transgenic Mouse Model of Alzheimer Amyloidosis. J. Neurosci. 22: 446-454 [Abstract] [Full Text]  
• Davies, S, Ramsden, D B (2001). Huntington's disease. Mol. Pathol. 54: 409-413 [Abstract] [Full Text]  
• Asthana, S., Baker, L. D., Craft, S., Stanczyk, F. Z., Veith, R. C., Raskind, M. A., Plymate, S. R. (2001). High-dose estradiol improves cognition for women with AD: Results of a randomized study. Neurology 57: 605-612 [Abstract] [Full Text]  
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• Chen, S., Wetzel, R. (2001). Solubilization and disaggregation of polyglutamine peptides. Protein Sci. 10: 887-891 [Abstract] [Full Text]  
• Chang, H. Y., Yang, X. (2000). Proteases for Cell Suicide: Functions and Regulation of Caspases. Microbiol. Mol. Biol. Rev. 64: 821-846 [Abstract] [Full Text]  
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• Wickner, S., Maurizi, M. R., Gottesman, S. (1999). Posttranslational Quality Control: Folding, Refolding, and Degrading Proteins. Science 286: 1888-1893 [Abstract] [Full Text]  
• Nemeroff, C. B. (1999). The Preeminent Role of Neuropeptide Systems in the Early Pathophysiology of Alzheimer Disease: Up With Corticotropin-Releasing Factor, Down With Acetylcholine. Arch Gen Psychiatry 56: 991-992 [Full Text]  
• Prasad, K. N., Cole, W. C., Kumar, B. (1999). Multiple Antioxidants in the Prevention and Treatment of Parkinson's Disease. J. Am. Coll. Nutr. 18: 413-423 [Abstract] [Full Text]  
• Shinder, G. A., Lacourse, M.-C., Minotti, S., Durham, H. D. (2001). Mutant Cu/Zn-Superoxide Dismutase Proteins Have Altered Solubility and Interact with Heat Shock/Stress Proteins in Models of Amyotrophic Lateral Sclerosis. J. Biol. Chem. 276: 12791-12796 [Abstract] [Full Text]  
• O'Nuallain, B., Wetzel, R. (2002). Conformational Abs recognizing a generic amyloid fibril epitope. Proc. Natl. Acad. Sci. USA 99: 1485-1490 [Abstract] [Full Text]  
• Muchowski, P. J., Schaffar, G., Sittler, A., Wanker, E. E., Hayer-Hartl, M. K., Hartl, F. U. (2000). Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proc. Natl. Acad. Sci. USA 97: 7841-7846 [Abstract] [Full Text]