Worm Breeder's Gazette 11(3): 44

These abstracts should not be cited in bibliographies. Material contained herein should be treated as personal communication and should be cited as such only with the consent of the author.

mec-4 and deg-1, Genes that can Mutate to Induce Neuronal Degeneration, are Highly Homologous

Monica Driscoll and Marty Chalfie

Neuronal degeneration in C.  elegans can be caused by mutations in 
two genes, mec-4 and deg-1.  Dominant alleles e1611, u214, and u231 of 
the mec-4 gene induce the degeneration of the touch receptor neurons.  
The dominant allele u38 of the deg-1 gene causes degeneration of a 
different group of neurons (including the PVC interneurons).  Although 
different cells are affected by these mutations, the deaths induced 
appear physically similar--characterized by a vacuolar appearance of 
the dying cells before their disappearance.  In addition, since 
mutations in the mec-6 gene suppress both mec-4 and deg-1 
degenerations, it appears that the mec-4 and deg-1 gene products act 
similarly to induce degeneration in different cells.  We are 
interested in deciphering the normal function of these gene products 
and in determining the nature of the changes that can culminate in 
abnormal cell death.  We report here that mec-4 and deg-1 are members 
of a gene family and encode highly homologous proteins.
The mec-4 gene was mapped to cosmid T20B9 by standard transposon 
tagging techniques.  A 4 kb EcoRI restriction fragment within this 
cosmid was shown to be the site of three transposon insertions that 
are absent in revertants.  In addition, a gamma ray-induced allele and 
an EMS-induced allele show polymorphisms in this fragment.  5x10+E6 
plaques from Chris Martin's cDNA library (2-3 kb size fraction) were 
screened and a single 1.6 kb cDNA that included the sequences covered 
by the polymorphisms was isolated.  We have sequenced this cDNA and 
the corresponding genomic DNA.  The cDNA contains an open reading 
frame that encodes a protein of 494 amino acids.  It contains a poly A 
tail, so we are confident that the 3' end of the gene is included in 
the cDNA clone.  The 494 amino acids are encoded in 12 exons in the 
genomic DNA.  We suspect that the mec-4 gene product may be larger 
than 494 amino acids since there are substantial open reading frames 
in the genomic sequence 5' to the position where the cDNA begins.  We 
are currently attempting to identify the real 5' end of the gene 
through RACE amplification experiments.
The deduced amino acid sequence of the mec-4 protein can be roughly 
divided into three domains.  The first 430 amino acids include many 
cys and pro residues.  In addition, this 'domain' has five sites for 
possible N-linked glycosylation.  The next 30 amino acids are largely 
hydrophobic and are likely to constitute a trans-membrane domain.  The 
carboxy terminal domain contains many charged residues.
Although the deduced mec-4 protein does not have extensive homology 
with any entries in the current GenBank database, the mec-4 protein 
exhibits striking homology to the deg-1 protein sequence.  These 
proteins are 50% identical over most of their lengths.  We have seen 
evidence of other cross-homologous C.  elegans genes on Southern blots.
About eight cross-homologous bands are seen using a mec-4 probe.  
Similar cross-homology has been reported for the deg-1 gene (Wolinsky 
and Chalfie, WBG 10(2), Chalfie and Wolinsky, Nature, in press).  Thus,
it appears that the C.  elegans genes that can mutate to cause 
degeneration are members of a gene family.
We have constructed libraries of genomic DNA from the three dominant 
mec-4 mutants and have isolated clones of the e1611, u214, and u231 
alleles.  We are currently sequencing these alleles to determine the 
amino acid changes that are associated with degeneration.  In addition,
we have isolated other cross-homologous genes from C.  elegans to 
continue our study of this gene family.