Neurochemical Changes in Leigh's Disease

Murphy, Jerome V.

doi:10.3177/jnsv.22.supplement_69

Cited by 7 publications

(2 citation statements)

References 10 publications

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“…The only sign of deficiency displayed by these lambs was anorexia and this correlates with the above findings in rats and in pigeons. The thiamin phosphate results are very different from the results on patients with Leigh's disease [also known as subacute necrotizing encephalopathy (SNE)], in which thiamin triphosphate levels decreased markedly and the diphosphate increased in affected areas of brain (Murphy, 1976). These results also differ from one report on thiamin-deficient rat brain, where the concentrations of thiamin and each of the esters were all significantly reduced by age 25 days (Muir, 1973).…”

Section: Discussioncontrasting

confidence: 68%

“…She found that thiamin phosphates in the brains of the mothers were less susceptible to depletion than in the young, and that the di-and triphosphates were maintained at a relatively high level at the expense of thiamin and thiamin monophosphate. Murphy (1976) found that in the brain of Leigh's disease patients the decrease in thiamin triphosphate correlated with severity of the lesion, and there was a concurrent increase in thiamin diphosphate. Loew (1974) has suggested that polioencephalomalacia (PEM) might be a suitable animal model for studying Leigh's disease, because of the similarity of the lesions and the clinical signs.…”

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confidence: 94%

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Thiamin Deficiency in the Lamb: Changes in Thiamin Phosphate Esters in the Brain

Thornber

Dunlop

Gawthorne

1980

Journal of Neurochemistry

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Concentration of thiamin (unphosphorylated), thiamin monophosphate (TMP), thiamin diphosphate (TDP), and thiamin triphosphate (TTP) were measured in three regions of the brain of seven pairs of lambs. The lambs were maintained on a thiamin-free synthetic diet for 2, 3, or 4 weeks. Controls were pair-fed and supplemented with thiamin. The three brain regions were: (1) dorso-lateral aspect of the cortex [common site for lesions of polioencephalomalacia (PEM)]; (2) pyriform lobe of the cortex (no PEM lesions are found here); (3) white matter of the internal capsule (no PEM lesions found here). The concentration of TTP ina ll three sections of brain was maintained at control values for up to 4 weeks on the thiamin-deficient diet. TDP concentration decreased to 22% of control values in both regions of grey matter after 4 weeks on the diet. Unphosphorylated thiamin and TMP decreased to a smaller extent than TDP.

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Section: Discussioncontrasting

confidence: 68%

mentioning

confidence: 94%

Thiamin Deficiency in the Lamb: Changes in Thiamin Phosphate Esters in the Brain

Thornber

Dunlop

Gawthorne

1980

Journal of Neurochemistry

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Studies on ATP

1978

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The experiments described in this paper serve as a contribution to the solution of the discrepancies which exist in the assay of ATP:thiamine diphosphate phosphotransferase activity (EC 2.7.4.15), presently in use as a tool for the diagnosis of Leigh's disease (SNE, subacute necrotizing encephalomyelopathy). The results obtained with this phosphotransferase assay can, in part, be explained by the presence of thiamine triphosphate (ThTP) in the preparation of thiamine diphosphate (ThDP) used as a substrate, by the inhibition by ATP of the ThTP phosphohydrolase activity, present in fractions of rat brain homogenates, and by the stimulation by ThDP of the ATPase activity. When [2(-14)C-thiazole]thiamine was used for the synthesis of [14C]ThTP in fractions of rat brain, it was found that after chromatographic separation of thiamine and its phosphates, 14C radioactivity could be demonstrated in the ThTP fractions, even in the absence of an enzyme source. Probably a complex is formed between [14C]thiamine and a phosphate ester which behaves chromatographically as ThTP. It is concluded that the assay system for the measurement of ThTP synthesis in its present form is, in our hands, not suitable for diagnostic purposes.

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Thiamine in excitable tissues: Reflections on a non-cofactor role

Bettendorff

1994

Metab Brain Dis

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Neurochemical Changes in Leigh's Disease

Cited by 7 publications

References 10 publications

Thiamin Deficiency in the Lamb: Changes in Thiamin Phosphate Esters in the Brain

Thiamin Deficiency in the Lamb: Changes in Thiamin Phosphate Esters in the Brain

Studies on ATP

Thiamine in excitable tissues: Reflections on a non-cofactor role

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