References of "Kish, Stephen J"
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See detailLow Thiamine Diphosphate Levels in Brains of Patients with Frontal Lobe Degeneration of the Non-Alzheimer's Type
Bettendorff, Lucien ULiege; Mastrogiacomo, Frank; Wins, Pierre et al

in Journal of Neurochemistry (1997), 69(5), 2005-2010

We compared the thiamine and thiamine phosphate contents in the frontal, temporal, parietal, and occipital cortex of six patients with frontal lobe degeneration of the non-Alzheimer's type (FNAD) or ... [more ▼]

We compared the thiamine and thiamine phosphate contents in the frontal, temporal, parietal, and occipital cortex of six patients with frontal lobe degeneration of the non-Alzheimer's type (FNAD) or frontotemporal dementia with five age-, postmortem delay-, and agonal status-matched control subjects. Our results reveal a 40-50% decrease in thiamine diphosphate (TDP) in the cortex of FNAD patients, whereas thiamine monophosphate was increased 49-119%. TDP synthesizing and hydrolyzing enzymes were unaffected. The activity of citrate synthase, a mitochondrial marker enzyme, was decreased in the frontal cortex of patients with FNAD, but no correlation with TDP content was found. These results suggest that decreased contents of TDP, which is essentially mitochondrial, is a specific feature of FNAD. As TDP is an essential cofactor for oxidative metabolism and neurotransmitter synthesis, and because low thiamine status (compared with other species) is a constant feature in humans, a nearly 50% decrease in cortical TDP content may contribute significantly to the clinical symptoms observed in FNAD. This study also provides a basis for a trial of thiamine, to improve the cognitive status of the patients. [less ▲]

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See detailBrain Levels of Thiamine and Its Phosphate Esters in Friedreich's Ataxia and Spinocerebellar Ataxia Type 1
Bettendorff, Lucien ULiege; Mastrogiacomo, Frank; LaMarche, J. et al

in Movement Disorders (1996), 11(4), 437-439

Decreased blood and cerebrospinal fluid levels of thiamine have been reported in patients with spinocerebellar ataxia disorders. To determine whether a thiamine deficiency is present in the brain, we ... [more ▼]

Decreased blood and cerebrospinal fluid levels of thiamine have been reported in patients with spinocerebellar ataxia disorders. To determine whether a thiamine deficiency is present in the brain, we measured levels of thiamine and its phosphate esters thiamine monophosphate (TMP) and thiamine diphosphate (TDP), in postmortem cerebellar and cerebral cortices of patients with Friedreich's ataxia (FA) and spinocerebellar ataxia type 1 (SCA1). Brain levels of free (nonphosphorylated) thiamine, TMP, TDP, and total thiamine in FA and SCA1 were, on average, not significantly different from control values. However, a nonsignificant trend was observed for slightly reduced levels of TDP and total thiamine in cerebellar cortex of the SCA1 patients, a finding that might be related to the severe neuronal damage in this brain area. We conclude that in FA, brain thiamine concentrations are normal, whereas in SCA1 the levels are, at most, only slightly reduced. [less ▲]

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See detailImmunoreactive levels of α-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1
Mastrogiacomo, Frank; LaMarche, Jacques; Dozic, Slobodan et al

in Neurodegeneration: A Journal for Neurodegenerative Disorders, Neuroprotection, and Neuroregeneration (1996), 5

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See detailThiamine, Thiamine Phosphates, and Their Metabolizing Enzymes in Human Brain
Bettendorff, Lucien ULiege; Mastrogiacomo, Frank; Kish, Stephen J et al

in Journal of Neurochemistry (1996), 66(1), 250-8

Total thiamine (the sum of thiamine and its phosphate esters) concentrations are two- to fourfold lower in human brain than in the brain of other mammals. There were no differences in the total thiamine ... [more ▼]

Total thiamine (the sum of thiamine and its phosphate esters) concentrations are two- to fourfold lower in human brain than in the brain of other mammals. There were no differences in the total thiamine content between biopsied and autopsied human brain, except that in the latter, thiamine triphosphate was undetectable. The main thiamine phosphate-metabolizing enzymes could be detected in autopsied brain, and the kinetic parameters were comparable to those reported in other species. Thiamine diphosphate levels were lowest in hippocampus (15 +/- 4 pmol/mg of protein) and highest in mammillary bodies (24 +/- 4 pmol/mg of protein). Maximal levels of thiamine and its phosphate ester were found to be present at birth. In parietal cortex and globus pallidus, mean levels of total thiamine in the oldest age group (77-103 years) were, respectively, 21 and 26% lower than those in the middle age group (40-55 years). Unlike cerebral cortex, the globus pallidus showed a sharp drop in thiamine diphosphate levels during infancy, with concentrations in the oldest group being only approximately 50% of the levels present during the first 4 months of life. These data, consistent with previous observations conducted in blood, suggest a tendency toward decreased thiamine status in older people. [less ▲]

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See detailBrain protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease
Mastrogiacomo, Frank; Lindsay, J. Gordon; Bettendorff, Lucien ULiege et al

in Annals of Neurology (1996), 39

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See detailBrain thiamine, its phosphate esters, and its metabolizing enzymes in Alzheimer's disease.
Mastrogiacomo, Frank; Bettendorff, Lucien ULiege; Grisar, Thierry ULiege et al

in Annals of Neurology (1996), 39

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