Huntingtin aggregates may not predict neuronal death in Huntington's disease

Annals of Neurology

Volumn 46, Issue 6, 1999, Pages 842-849

  Kuemmerle, Stefan ^a,c,d   Gutekunst, Claire Anne ^e   Klein, Autumn M ^a,c   Li, Xiao Jiang ^e   Li, Shi Hua ^e   Beal, M Flint ^b,f   Hersch, Steven M ^e   Ferrante, Robert J ^a,c  

^a BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b MASSACHUSETTS GENERAL HOSPITAL   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d TECHNICAL UNIVERSITY OF MUNICH   (Germany)

^e EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^f WEILL CORNELL MEDICAL COLLEGE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

HUNTINGTIN; POLYGLUTAMIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE PHOSPHATE DEHYDROGENASE; CALBINDIN; DIHYDROLIPOAMIDE DEHYDROGENASE; HD PROTEIN, HUMAN; NERVE PROTEIN; NUCLEAR PROTEIN;

ARTICLE; BRAIN CORTEX; CELL LOSS; CELL PROTECTION; CONTROLLED STUDY; CORPUS STRIATUM; FEMALE; HUMAN; HUMAN TISSUE; HUNTINGTON CHOREA; MALE; NERVE CELL DEGENERATION; NERVE CELL NECROSIS; NEUROTOXICITY; PERIKARYON; PRIORITY JOURNAL; ADOLESCENT; ADULT; AGED; BRAIN DISEASE; CELL NUCLEUS; IMMUNOHISTOCHEMISTRY; MIDDLE AGED; NERVE CELL; PATHOLOGY; REFERENCE VALUE;

ADOLESCENT; ADULT; AGED; AGED, 80 AND OVER; BRAIN DISEASES; CALCIUM-BINDING PROTEIN, VITAMIN D-DEPENDENT; CELL NUCLEUS; CEREBRAL CORTEX; CORPUS STRIATUM; DIHYDROLIPOAMIDE DEHYDROGENASE; HUMANS; HUNTINGTON DISEASE; IMMUNOHISTOCHEMISTRY; MIDDLE AGED; NERVE TISSUE PROTEINS; NEURONS; NUCLEAR PROTEINS; REFERENCE VALUES;

EID: 0032590053     PISSN: 03645134     EISSN: None     Source Type: Journal    
DOI: 10.1002/1531-8249(199912)46:6<842::AID-ANA6>3.0.CO;2-O     Document Type: Article

References (31)

1. Kowall NW, Ferrante RJ. Huntington's disease. In: Markesbery M, ed. Neuropathology of dementing disorders. London: Edward Arnold, 1998:219-256
2. Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72: 971-983
3. Gusella JF, MacDonald ME. Huntington's disease. Semin Cell Biol 1995;6:21-28
4. Plassart E, Fontaine B. Genes with triplet repeats: a new class of mutations causing neurological diseases. Biomed Pharmacother 1994;48:191-197
5. Ross CA. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neu rodegenerative diseases? Neuron 1997;19:1147-1150
6. La Spada AR, Paulson HL, Fischbeck KH. Trinucleotide repeat expansion in neurological disease. Ann Neurol 1994;36:814-822
7. Cooper AJ, Sheu KF, Burke JR, et al. Pathogenesis of inclusion bodies in (CAG)n/Qn-expansion diseases with special reference to the role of tissue transglutaminase and to selective vulnerability. J Neurochem 1999;72:889-899
8. Hersch S, Ferrante RJ. Neuropathology and pathophysiology of Huntington's disease. In Watts RL, Koller WC, eds. Movement disorders: neurologic principles and practice. New York: McGraw-Hill, 1997:503-526
9. Albin RL, Reiner A, Anderson KD, et al. Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia. Ann Neurol 1990;27:357-365
10. Ferrante RJ, Kowall NW, Beal MF, et al. Selective sparing of a class of striatal neurons in Huntington's disease. Science 1985; 230:561-563
11. Ferrante RJ, Beal MF, Kowall NW, et al. Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease. Brain Res 1987;411:162-166
12. Ferrante RJ, Kowall NW, Richardson EP Jr. Proliferative and degenerative changes in striatal spiny neurons in Huntington's disease: a combined study using the section-Golgi method and calbindin D28k immunocytochemistry. J Neurosci 1991;11: 3877-3887
13. Ferrante R, Gutekunst C-A, Persichetti F, et al. Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum. J Neurosci 1997;17:3052-3063
14. Kosinski CM, Cha JH, Young AB, et al. Huntingtin immunoreactivity in the rat neostriatum: differential accumulation in projection and interneurons. Exp Neurol 1997;144:239-247
15. Roizin RAC, Stellar S, Liu JC. Neuronal nuclear-cytoplasmic changes in Huntington's chorea: electron microscope investigations. Adv Neurol 1979;23:95-122
16. Becher MW, Kotzuk JA, Sharp AH, et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998; 4:387-397
17. Davies SW, Turmaine M, Cozens BA, et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997;90: 537-548
18. Cooper J, Schilling G, Peters M, et al. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum Mol Genet 1998;7:783-790
19. Martindale D, Hackam A, Wieczorek A. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet 1998;18:150-154
20. DiFiglia M, Sapp E, Chase KO, et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997;277:1990-1993
21. Gutekunst C-A, Li S-H, Yi H, et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci 1999;19:2522-2534
22. Klement I, Skinner P, Kaytor M. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 1998;95:41-53
23. Saudou F, Finkbeiner S, Devys D, et al. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998;95:55-66
24. Ordway JM, Tallaksen-Greene S, Gutekunst CA, et al. Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 1997;91:753-763
25. Vonsattel JP, Myers RH, Stevens TJ, et al. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 1985;44:559-577
26. Cudkowicz M, Kowall NW. Degeneration of pyramidal projection neurons in Huntington's disease cortex. Ann Neurol 1990; 27:200-204
27. Davies SW, Beardsall K, Turmaine M, et al. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet 1998;351:131-133
28. Kim M, Lee HS, LaForet G, et al. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J Neurosci 1999; 19:964-973
29. Huynh DP, Del Bigio MR, Ho DH, Pulst SM. Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2. Ann Neurol 1999;45:232-241
30. Sisodia SS. Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial? Cell 1998;95: 1-4
31. Johnston JA, Ward CL, Kopito RR. Aggresomes: a cellular response to misfolded proteins. J Cell Biol 1998;143:1883-1898