Abstract
The dominant mutation deg-1(u38) results in a toxic gene product that leads to the late-onset degeneration of a small number of neurons in the nematode Caenorhabditis elegans. Both intragenic and extragenic mutations as well as changes in wild-type gene dosage can delay or block the time of onset of the neuronal deaths. The deg-1 gene has been cloned and a partial complementary DNA reveals that the gene encodes a novel protein that may act as a membrane receptor. Because the late-onset loss of specific sets of neurons, often as a result of dominant mutations, is characteristic of several human neurodegenerative diseases, the analysis of the deg-1 gene and its suppressors may provide a means of understanding the mechanisms underlying some of these human diseases.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 51 print issues and online access
$199.00 per year
only $3.90 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
1. Adams R D & Victor M in Principles of Neurology 3rd edn Ch 42 859-901 (McGraw-Hill New York 1985) 2. Hayden M R Huntington s Chorea (Springer Berlin 1981) 3. Gusella J F et al Nature 306, 234-238 (1983) 4. Mulder D W Kurland L T Offord K P & Beard M Neurology 36, 511-517 (1986) 5. Davies P Neurobiol Aging 7, 459 466 (1986) 6. Harding A E in Hereditary Ataxias and Related Disorders Cb 10 129-165 (Churchill Livingstone Edinburgh 1984) 7. Chalfie, M & Au M Science 243,1027-1033 (1989) 8. Chalfie M & Sulston, J Devi Biol 82, 358-370 (1981) 9. Chalfie, M et al J Neurosci 5, 956-964 (1985) 10. Sulston, J E, Schierenberg, E, White, J G & Thomson, J N Devi Biol 100, 64-119 (1983) 11. Sulston, J E & Horvitz, H R Devi Biol 56, 110-156 (1977) 12. Herman, R K Genetics 116, 377-388 (1987) 13. Way, J C & Chalfie, M Genes Dev 3, 1823-1833 (1989) 14. Brenner, S Genetics 77, 71-94 (1974) 15. Greenwald, l S & Horvitz, H R Genetics 96,147-164 (1980) 16. Collms J, Saari, B & Anderson, P Nature 328, 726-728 (1987) 17. Suzuki, D T Science 170, 695-706 (1970) 18. Ferguson, E L & Horvitz H R Genetics 123, 109-121 (1989) 19. Elhs, H M & Horvitz, H R Cell 44, 817-829 (1986) 20. Avery, L & Horvitz, H R Cell 51,1071-1078 (1987) 21. Choi D Neuron l, 623-634 (1988) 22. White, J G, Albertson, D G & Anness, M A Nature 271, 764-766 (1978) 23. Sulston, J E & Horvitz, H R Devi Biol 82, 41-55 (1981) 24. Coulson, A, Sulston, J, Brenner, S & Karn, J Proc natn Acad Sei U S A 83, 7821-7825 (1986) 25. Savage, C et ai Genes Dev 3, 870-881 (1989) 26. Hubbard, S C & Ivatt, R J A Rev Biochem 50, 555-583 (1981) 27. Adams, G A & Rose J K Cell 41, 1007-1015 (1985) 28. Yanagi, Y ef ai Nature 308,146-149 (1984) 29. Hedrick, S M, Nielsen, E A, Kavaler, J, Coh鮠D l & Davis M M Nature 308,153-158 (1984) 30. Yamamoto, T ef ai Cell 39, 27-38 (1984) 31. Ullrich, A et ai Nature 309, 418-425 (1984) 32. Wen, D ef ai Biochemistry 26, 4350-4357 (1987) 33. Radeke, M J, Misko, T P Hsu, C, Herzenberg, L A & Shooter, E M Nature 325, 593-597 (1987) 34. Trent, C, Tsung, N & Horvitz, H R Genetics 104, 619-647 (1983) 35. Ferguson, E L & Horvitz, H R Genetics 110,17-72 (1985) 36. Folstem, S E ef ai Science 229, 776-779 (1985) 37. Leonard, J P & Salpeter, M M J Cell Biol 82, 811-819 (1979) 38. Hogan, P G J Neurosci (in the press) 39. Choi, D W J Neurosci 7, 369-379 (1988) 40. Takumi, T, Ohkubo H & Nakanishi, S Science 242,1042-1045 (1988) 41. Murai, T, Kakizuka, A, Takumi, T, Ohkubo, H & Nakanishi, S Biochem biophys RCommun 161, 176-181 (1989) 42. Kang J Lemaire H-G Unterbeck.A Salbaum J M & Masters C L Nature 325,733-736 (1987) 43. McCusick V A Mendelian Inheritance in Man 8th edn (Johns Hopkms University Press, Baltimore 1989) 44. Dickson, D W ef ai Acta neuropath 71, 216-223 (1986) 45. Kohn R J Neurol Neurosurg Psychiat 34, 427-431 (1971) 46. Reif-Kohn, R & Ml, G ConfNeurol 36, 23-32 (1974) 47. Duchen, L W & Strich, S J J Neurol Neurosurg Psychiat 31, 535-542 (1968) 48. Caddy K W T & Briscoe, T J Bram R91, 276-280 (1975) 49. LaVail, M M, Blanks, J C & Mullen, R J J comp Neurol 212, 217-230 (1982) 50. Pilar G & Landmesser L J Cell Biol 68, 339-356 (1976) 51. Chu-Wang. l -W & Oppenheim, R W J comp Neurol 177, 33-58 (1978) 52. Knyihar, E, Csilhk, B & Rakic, P Science 202,1206-1209 (1978) 53. Cunnmgham T J Int Rev Cytol 74, 163-186 (1982) 54. Valverde, F & Facal-Valverde, M V J comp Neurol 269,168-192 (1988) 55. Chun J J M & Shatz C J J comp Neurol 282, 555-569 (1989) 56. Way J C & Chalfie, M Cell 54, 5-16 (1988) 57. Hedgecock E M, Sulston, J E & Thomson, J N Science 220,1277-1279 (1983) 58. Chalfie M & Thomson, J N J Cell Biol 82, 278-289 (1979) 59. Ward, S, Thomson, N, White, J G & Brenner S J comp Neurol 160, 313-337 (1975) 60. White, J G, Southgate E, Thomson, J N & Brenner S Phil Trans R Soe B314,1-340 (1986) 61. Emmons S C Klass, M R & Hirsh D Proc natn Acad Sei USA 76,1333-1337 (1979) 62. Maniatis T, Fritsch, E F & Sambrook, J Molecular Cloning A Laboratory Manual (Cold Spring Harbor Laboratory New York 1982) 63. Ish-Horowicz, D & Burke J F Nucleic Acids Res 9, 2989-2998 (1981) 64. Fire, A EMBO J 5, 2673-2680 (1986) 65. Ozkaynak, E & Putney, S D BioTechniques 5, 770-773 (1987) 66. Sanger, F, Nicklen, S & Coulson, A R Proc natn Acad Sei USA 74, 5463-5467 (1977)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Chalfie, M., Wolinsky, E. The identification and suppression of inherited neurodegeneration in Caenorhabditis elegans. Nature 345, 410–416 (1990). https://doi.org/10.1038/345410a0
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1038/345410a0
This article is cited by
-
A guide to cell death pathways
Nature Reviews Molecular Cell Biology (2023)
-
Pore architecture and ion sites in acid-sensing ion channels and P2X receptors
Nature (2009)
-
Mutant Sodium Channel for Tumor Therapy
Molecular Therapy (2009)
-
The C. elegansEMAP-like protein, ELP-1 is required for touch sensation and associates with microtubules and adhesion complexes
BMC Developmental Biology (2008)
Comments
By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.
