MOLECULAR-GENETICS OF HUNTINGTONS-DISEASE

Huntington's disease is an inherited disorder in which selective neuro nal loss in the brain leads to a characteristic choreic movement disor der. The successful mapping of the Huntington's disease gene to chromo some 4 set off a torrent of similar studies in other inherited disorde rs as investigators attempted to locate and isolate human disease gene s with this new approach. Although it took a decade-long quest since t he initial mapping of the genetic defect, the gene causing Huntington' s disease has recently been isolated. Discovery of the mutational mech anism causing Huntington's disease has explained some of the peculiari ties of inheritance of this intriguing disorder and creates hope for a better understanding of the cause of neuronal cell death that could e ventually lead to a treatment.