Huntingtons Disease

It is often difficult to determine the exact time of onset of Huntington=s disease as it comes on subtly, usually between the ages of 35 and 40 (1). During the early stages of Huntington=s disease, spontaneous abnormal movements can often be blended with normal movements so they are less noticeable, but with time, the abnormal movements become more obvious. With time, they come to involve the entire body, and make the simplest tasks such as dressing and eating almost impossible without aid. Distinct changes can be seen in the brain with computed tomography (a CAT scan). Mental changes also occur - mild at first, but increasing in severity over time. Patients become irritable and excitable, and gradually lose interest in normal activities.

As the disease progresses, Huntington=s patients may behave irresponsibly, wander aimlessly, and even lose control over their impulses a

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nitrous oxide in Huntington=s disease analogous to its postulated role in Duchenne muscular dystrophy. An N(H.sub 2)-terminal fragment of Huntington was localized to neuronal intranuclear inclusions and dystrophic neurites in the cortex and striatum of Huntington=s disease patients, and polyglutamine length was found to influence the extent of Huntington accumulation in these structures by DiFiglio et al (5). Ubiquitin was also found in the intranuclear inclusions and dystrophic neurites, which suggests that abnormal Huntington is targeted for proteolysis but is resistant to removal. The aggregation of mutant Huntington may be part of its pathogenic mechanism in Huntington=s disease, as suggested previously (12). Le, Fung, and Trent (6) used capillary electrophoresis to analyze the DNA mutation in Huntington=s disease. The technique appeared useful in testing for Huntington=s, but sizing of the CAG repeat was a problem because the polymers used in this form of electrophoresis are still in the developmental phase. ELECTROPHORESIS METHODS There are many forms of electrophoresis which can be used to separate molecules of various sizes and to identify proteins (6). Free-zone electrophoresis passes a current through molecu
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