Huntington disease: normal lipid composition of purified neuronal perikarya and whole cortex

This is the first report of the lipid composition of human neurons. Neuronoal perikarya were isolated from frozen samples of the cerebral cortex of persons with Huntington disease and two normal controls. These were analyzed for total lipid, individual lipids, and gangliosides. No differences were detected between diseased and normal cells. In addition, gray matter samples from the same patients, and one additional patient and control sample, were analyzed and found not to differ. Thus the ultrastructural abnormalities seen in cortical biopsies are not reflected in the concentration of the major lipid classes.