Thiamin mono- and pyrophosphatase activities from brain homogenate of Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia patients

Umberto Laforenza, Cesare Patrini, Marco Poloni, Paolo Mazzarello, Mauro Ceroni, D. Carleton Gajdusek, Ralph M. Garruto

Research output: Contribution to journalArticlepeer-review

Abstract

Thiamin-pyrophosphatase (TPPase) and thiamin-monophosphatase (TMPase) were determined using a spectrophotometric method at various pH values (5.5, 7.5, and 9.0) in brain tissue obtained at autopsy from amyotrophic lateral sclerosis (ALS) and parkiinsonism-dementia (PD) patients from Guamaand from Guamanian patients who died from other diseases (controls). TPPase separation by thin-layer polyacrylamide gel isoelectric focusing (IEF) was also performed using both gray and white matter. TPPase content, chemically determined at pH 9.0, was found to be significantly reduced in the frontal cortex of ALS and PD patients compared to controls TMPase content, on the contrary, was unchanged. IEF analysis showed 9 clear-cut bands with TPPase activity in the pH range 5.4-7.2 and a broad band at pH 4.7-5.2. The enzymatic activity was higher in gray than in white matter. In one patient the pattern was clearly different, with two additional bands observed at pH 7.1 and 6.7, and thought to be due to genetic microheterogeneity.

Original languageEnglish
Pages (from-to)156-161
Number of pages6
JournalJournal of the Neurological Sciences
Volume109
Issue number2
DOIs
Publication statusPublished - 1992

Keywords

  • Guamanian amyotrophic lateral sclerosis
  • Isoelectric focusing
  • Parkinsonism-dementia
  • Thiamin monophosphatase
  • Thiamin pyrophosphatase

ASJC Scopus subject areas

  • Ageing
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

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