DECREASED PLASMA ALANINE AND ISOLEUCINE IN HUNTINGTONS-DISEASE

Amino acid concentrations in plasma of patients with Huntington's dise ase (HD) were determined in 16 patients and 21 age- and sex-matched he althy controls. Alanine and isoleucine were significantly decreased in HD plasma whereas arginine, histidine, leucine, lysine, ornithine, pr oline, serine, threonine, tyrosine, and valine showed no significant c hanges. Our findings confirm the decreases of alanine and isoleucine t hat were described in plasma and cerebrospinal fluid by other investig ators. A possible defect in cellular uptake or metabolism of neutral a mino acids seems to be a consistent feature of HD.