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Science 4 April 1969:
Vol. 164. no. 3875, pp. 74 - 75
DOI: 10.1126/science.164.3875.74
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Articles

Tbiamine-Triphosphate Deficiency in Subacute Necrotizing Encephalomyelopathy

Jack R. Cooper 1, Yoshinori Itokawa 1, and Jonathan H. Pincus 1

1 Department of Pharmacology and Section of Neurology, Yale University School of Medicine, New Haven, Connecticut

Extracts of tissue fluids from a patient with subacute necrotizing encephalomyelopathy inhibit thiamine pyrophosphate-adenosine triphosphate phosphotransferase of rat brain. Brain tissue from the patient, in contrast to normal brain tissue, contained essentially no thiamine triphosphate, although thiamine and its other phosphate esters were present in normal concentrations. These findings suggest a relation between this disease and thiamine triphosphate.


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Leigh Disease: Biochemical Characteristics of the Inhibitor.
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Arch Neurol 25, 449-460
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Neuro-ophthalmology.
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Thiamine Release from Nerve Membranes by Tetrodotoxin.
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Science 166, 759-760
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