Studies on ATP

Schrijver, J; Dias, Th.; Hommes, F. A.

doi:10.1007/bf00965993

Cited by 14 publications

(2 citation statements)

References 28 publications

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“…Of particular interest are the existence and function of thiamine triphosphate (TTP) and its presence in several animal tissues has been reported previously (2)(3)(4)(5)(6). However, the method of determining TTP itself is still a subject of controversy in the study of enzymatic TTP synthesis (7).…”

Section: Discussionmentioning

confidence: 99%

Concentrations of thiamine and its phosphate esters in rat tissues determined by high-performance liquid chromatography.

Ishii¹,

Sarai²,

Sanemori

et al. 1979

Journal of Nutritional Science and Vitaminology, J Nutr Sci Vit

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SummaryThiamine and its phosphate esters in rat tissues were determined by high-performance liquid chromatography after conversion to thiochrome and its phosphate esters as described previously (Ishii, K. et al. (1979) Anal. Blochem., 97, 191-195). Total thiamine concentrations determined in the brain, liver, heart and kidney were 6.39, 25.2, 15.7, and 14.9nmol/g wet weight, respectively. In all the tissues tested, thiamine, its monophosphate, pyrophosphate, and triphosphate were found to be present at the rates of 1.2-7.7, 5.9-11.0, 85.7-90.0, and 0.7-1.6%, respectively. Although these percentages of thiamine phosphates were similar to those reported previously using different procedures, the percentage of thiamine triphosphate content in rat tissues was found to be fairly lower than that reported. The time required for the whole process of analysis averaged 90min after isolation of the organs from the animal.

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Section: Discussionmentioning

confidence: 99%

Concentrations of thiamine and its phosphate esters in rat tissues determined by high-performance liquid chromatography.

Ishii¹,

Sarai²,

Sanemori

et al. 1979

Journal of Nutritional Science and Vitaminology, J Nutr Sci Vit

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show abstract

“…There are only a few studies on the distribution of these compounds in animal tissues [22], [42] and only two (from the same group) in humans showing decreased ThTP levels in the postmortem brains of patients with subacute necrotizing encephalomyelopathy (Leigh's disease) [43], [44]. However, the compound measured in the latter study may not have been authentic ThTP [45]. Indeed, ThTP measurements were unreliable before the development of HPLC techniques and we were unable to detect ThTP in human postmortem brains [18], [46], probably because of hydrolysis during the postmortem delay.…”

Section: Introductionmentioning

confidence: 95%

Thiamine Status in Humans and Content of Phosphorylated Thiamine Derivatives in Biopsies and Cultured Cells

et al. 2010

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BackgroundThiamine (vitamin B1) is an essential molecule for all life forms because thiamine diphosphate (ThDP) is an indispensable cofactor for oxidative energy metabolism. The less abundant thiamine monophosphate (ThMP), thiamine triphosphate (ThTP) and adenosine thiamine triphosphate (AThTP), present in many organisms, may have still unidentified physiological functions. Diseases linked to thiamine deficiency (polyneuritis, Wernicke-Korsakoff syndrome) remain frequent among alcohol abusers and other risk populations. This is the first comprehensive study on the distribution of thiamine derivatives in human biopsies, body fluids and cell lines.Methodology and Principal FindingsThiamine derivatives were determined by HPLC. In human tissues, the total thiamine content is lower than in other animal species. ThDP is the major thiamine compound and tissue levels decrease at high age. In semen, ThDP content correlates with the concentration of spermatozoa but not with their motility. The proportion of ThTP is higher in humans than in rodents, probably because of a lower 25-kDa ThTPase activity. The expression and activity of this enzyme seems to correlate with the degree of cell differentiation. ThTP was present in nearly all brain and muscle samples and in ∼60% of other tissue samples, in particular fetal tissue and cultured cells. A low ([ThTP]+[ThMP])/([Thiamine]+[ThMP]) ratio was found in cardiovascular tissues of patients with cardiac insufficiency. AThTP was detected only sporadically in adult tissues but was found more consistently in fetal tissues and cell lines.Conclusions and SignificanceThe high sensitivity of humans to thiamine deficiency is probably linked to low circulating thiamine concentrations and low ThDP tissue contents. ThTP levels are relatively high in many human tissues, as a result of low expression of the 25-kDa ThTPase. Another novel finding is the presence of ThTP and AThTP in poorly differentiated fast-growing cells, suggesting a hitherto unsuspected link between these compounds and cell division or differentiation.

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Cytochrome c oxidase deficiency in leigh syndrome

DiMauro

Servidei

Zeviani

et al. 1987

Annals of Neurology

354

158

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We studied 6 mitochondrial enzymes in crude extracts and isolated mitochondria from 5 children with pathologically proven subacute necrotizing encephalomyelopathy (Leigh syndrome). Samples were taken from brain (5 patients), skeletal muscle (4 patients), liver (4 patients), kidney (4 patients), heart (1 patient), and cultured fibroblasts (3 patients). An isolated defect of cytochrome c oxidase (COX) activity was found in brain (decrease of activity to 15 to 39% of the normal mean), muscle (9 to 20%), kidney (1 to 67%), and in the 1 available heart (4%) from a patient with cardiopathy. COX activity was also decreased in liver of 3 patients (2 to 13% of normal) and in cultured fibroblasts of 2 patients (18 and 27%), but it was normal in both liver and fibroblasts from 1 patient. Immunotitration using polyclonal antibodies against human heart COX showed essentially normal amounts of cross-reacting enzyme protein in various tissues from different patients. Electrophoresis of COX immunoprecipitated from brain mitochondrial extracts showed normal patterns of COX subunits in 2 patients. This study confirms the theory that COX deficiency is an important cause of Leigh syndrome.

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Studies on ATP

Cited by 14 publications

References 28 publications

Concentrations of thiamine and its phosphate esters in rat tissues determined by high-performance liquid chromatography.

Concentrations of thiamine and its phosphate esters in rat tissues determined by high-performance liquid chromatography.

Thiamine Status in Humans and Content of Phosphorylated Thiamine Derivatives in Biopsies and Cultured Cells

Cytochrome c oxidase deficiency in leigh syndrome

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