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
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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