Brain levels of thiamine and its phosphate esters in Friedreich's ataxia and spinocerebellar ataxia type 1
Autor: | Slobodan Dozic, Jacques Lamarche, Lucien Bettendorff, Stephen J. Kish, Frank Mastrogiacomo |
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Rok vydání: | 1996 |
Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Spinocerebellar Ataxia Type 1 Ataxia Adolescent Central nervous system disease 03 medical and health sciences chemistry.chemical_compound Cerebellar Cortex 0302 clinical medicine Cerebrospinal fluid Reference Values Internal medicine medicine Humans Thiamine Child 030304 developmental biology Spinocerebellar Degenerations Cerebral Cortex 0303 health sciences food and beverages Thiamine monophosphate Middle Aged medicine.disease Thiamine Monophosphate nervous system diseases 3. Good health Endocrinology Neurology chemistry Friedreich Ataxia Cerebellar cortex Child Preschool Spinocerebellar ataxia Female Neurology (clinical) medicine.symptom Thiamine Pyrophosphate human activities Neuroscience 030217 neurology & neurosurgery |
Zdroj: | Movement disorders : official journal of the Movement Disorder Society. 11(4) |
ISSN: | 0885-3185 |
Popis: | 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. |
Databáze: | OpenAIRE |
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