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Huntington's Disease — Imbalance of free amino acids in the cerebrospinal fluid of patients and offspring at-risk

✍ Scribed by G. Oepen; H. Cramer; R. Bernasconi; P. Martin


Publisher
Springer-Verlag
Year
1982
Tongue
English
Weight
559 KB
Volume
231
Category
Article
ISSN
1433-8491

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✦ Synopsis


A total of 27 different amino acids were determined in the fasting, morning lumbar CSF of 12 patients with Huntington's Disease (HD), 8 at-risk offspring and 16 non-choreic control patients. A significant (P less than 0.001) decrease was observed for asparagine, isoleucine, leucine, phenylalanine, histidine, arginine, alpha-aminoadipic acid and homocarnosine in patients with HD compared to the non-choreic controls. Only tyrosine was increased in HD. These alterations were to an extent more pronounced in 5 neurophysiologically conspicuous offspring. The alterations suggest that amino acid imbalance is an early metabolic disturbance in HD.


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