atrophy gene fromthe Huntington's disease locus

dentatorubropallidoluysian atrophy (DRPLA)isan autosomal dominant neurodegen- erative disorder. Clinical andgenetic findings in hereditary DRPLA are very similar tothoseof Huntington's disease (HD).However,itcan be differentiated fromHD bythepathological findings ofdentatorubral andpaliidoluysian atrophies and bya lackofprominent atrophy ofthestriatum at necropsy. Thehereditary DRPLA gene hasnot beenlocalised andthepossibility thatthetwo disease loci are alielic hasbeensuggested. We havesearched forlinkage between thelocus forhereditary DRPLA andD4S1OusingtheG8 probe, whichisa genetic markerlinked toHD.In fourfamilies, there were negative scores atall recombination fractions andthelodscore was