Free amino acid concentrations in spinal tissue from patients dying of motor neuron disease

Publisher Website
Google Scholar
Abstract
Because some investigators have reported abnormal concentrations of amino acids (AA) in fluids and tissues of patients with motor neuron disease (MND), the AA content of frozen anterior horn spinal cord tissue taken from 7 patients dying of MND was examined and compared with the results with those found in 12 control patients. NH3 (21 .+-. 8.1 vs. 12.7 .+-. 6.9 .mu.mol/g, P = 0.036) and ornithine (0.41 .+-. 0.3 vs. 0.16 .+-. 0.09, P = 0.036) were elevated in spinal tissue of MNP patients. Correlation analysis showed NH3 levels inversely related to duration of illness (r = -0.714, P = 0.036). Metabolic abnormalities therefore exist in MND. NH3 and ornithine may be adversely affecting motor neuron function, or, alternatively, they could be metabolic markers of a more generalized energy-deficient state in MND.