The identification and suppression of inherited neurodegeneration in Caenorhabditis elegans

Nature. 1990 May 31;345(6274):410-6. doi: 10.1038/345410a0.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Age Factors
  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Caenorhabditis / genetics
  • Caenorhabditis / growth & development*
  • Cloning, Molecular
  • Gene Expression
  • Genes
  • Genes, Dominant
  • Membrane Proteins / genetics
  • Molecular Sequence Data
  • Nerve Degeneration / genetics*
  • Phenotype
  • Suppression, Genetic


  • Membrane Proteins