Literature DB >> 8930410

Brain pyruvate oxidation in experimental thiamin-deficiency encephalopathy.

P Munujos1, J Coll-Cantí, J Beleta, F González-Sastre, F J Gella.   

Abstract

Pyrithiamine-induced thiamin deficiency has been used in rat as an experimental form of Wernicke-Korsakoff encephalopathy, a disease associated with chronic alcoholism. Although the main etiological factor is known to be the lack of thiamin, the biochemical mechanisms involved in the pathogenesis remain unclear. Thiamin-dependent enzymes were studied in brain mitochondria: alpha-ketoglutarate dehydrogenase activity exhibited 40% reduction, whereas pyruvate dehydrogenase did not change significantly. Polarographic recordings of mitochondrial respiration revealed a decreased State 3, when using pyruvate/malate, alpha-ketoglutarate or glutamine as a substrate, but the respiration rates remained unchanged with glutamate or succinate. This fall in pyruvate oxidation may be due to the impairment of alpha-ketoglutarate dehydrogenase, which follows pyruvate dehydrogenase in the metabolic pathway. A time course of lactate concentration showed dramatic increases in thalamus, mid brain, hypothalamus and colliculli, consistent with the anatomopathological findings. No increases were found before the onset of neurological symptoms.

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Year:  1996        PMID: 8930410     DOI: 10.1016/0009-8981(96)06385-1

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


  4 in total

1.  Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia.

Authors:  Marjorie J Lindhurst; Giuseppe Fiermonte; Shiwei Song; Eduard Struys; Francesco De Leonardis; Pamela L Schwartzberg; Amy Chen; Alessandra Castegna; Nanda Verhoeven; Christopher K Mathews; Ferdinando Palmieri; Leslie G Biesecker
Journal:  Proc Natl Acad Sci U S A       Date:  2006-10-11       Impact factor: 11.205

Review 2.  Pyruvate dehydrogenase complex: metabolic link to ischemic brain injury and target of oxidative stress.

Authors:  Erica Martin; Robert E Rosenthal; Gary Fiskum
Journal:  J Neurosci Res       Date:  2005 Jan 1-15       Impact factor: 4.164

3.  Glucose loading precipitates focal lactic acidosis in the vulnerable medial thalamus of thiamine-deficient rats.

Authors:  Darren Navarro; Claudia Zwingmann; Nicolas Chatauret; Roger F Butterworth
Journal:  Metab Brain Dis       Date:  2007-11-22       Impact factor: 3.584

4.  Novel mutations in IBA57 are associated with leukodystrophy and variable clinical phenotypes.

Authors:  Alessandra Torraco; Anna Ardissone; Federica Invernizzi; Teresa Rizza; Giuseppe Fiermonte; Marcello Niceta; Nadia Zanetti; Diego Martinelli; Angelo Vozza; Daniela Verrigni; Michela Di Nottia; Eleonora Lamantea; Daria Diodato; Marco Tartaglia; Carlo Dionisi-Vici; Isabella Moroni; Laura Farina; Enrico Bertini; Daniele Ghezzi; Rosalba Carrozzo
Journal:  J Neurol       Date:  2016-10-26       Impact factor: 4.849

  4 in total

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