Amino acids in autopsied human spinal cord. Selective changes in Friedreich's ataxia.

The distribution of glycine, glutamate, aspartate, glutamine, and taurine was measured at autopsy in 10 normal human spinal cords, and in 4 spinal cords from Friedreich's Ataxia patients, using a sensitive double-isotope microassay of their dansyl derivatives. Transverse sections of spinal cord from cervical, thoracic, and lumbar levels were dissected to afford samples of gray matter, posterior columns, dorsal white matter, and ventral white matter. Levels of glycine, glutamate, and glutamine were found to be elevated in lumbar gray matter, being 2-3 times higher than those found in white matter structures. Aspartate and taurine, on the other hand, were found to be distributed more evenly in autopsied human spinal cord. Selective abnormalities of amino acid distribution in Friedreich's Ataxia included decreased glutamate and glutamine in lumbar gray matter and posterior columns and increased taurine content of lumbar spinal cord. These changes may be of pathophysiological significance in this hereditary neurodegenerative disease.