α-ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors

Cláudia Funchal, Priscila De Lima Pelaez, Samanta Oliveira Loureiro, Lilian Vivian, Franciele Dall Bello Pessutto, Lúcia Maria Vieira De Almeida, Susana Tchernin Wofchuk, Moacir Wajner, Regina Pessoa Pureur*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

26 Scopus citations

Abstract

In this study we investigated the effects of α-ketoisocaproic acid (KIC), the main keto acid accumulating in the inherited neurometabolic disorder maple syrup urine disease (MSUD), on the in vitro incorporation of 32P into intermediate filament (IF) proteins from cerebral cortex of rats during development. KIC decreased the in vitro incorporation of 32P into the IF proteins studied up to day 12, had no effect on day 15, and increased this phosphorylation in cortical slices of 17- and 21-day-old rats. A similar effect on IF phosphorylation was achieved along development by incubating cortical slices with glutamate. Furthermore, the altered phosphorylation caused by the presence of KIC in the incubation medium was mediated by the ionotropic receptors NMDA, AMPA and kainate up to day 12 and by NMDA and AMPA in tissue slices from 17- and 21-day-old rats. The results suggest that alterations of IF phosphorylation may be associated with the neuropathology of MSUD.

Original languageEnglish (US)
Pages (from-to)267-276
Number of pages10
JournalDevelopmental Brain Research
Volume139
Issue number2
DOIs
StatePublished - Dec 15 2002

Keywords

  • Cytoskeleton
  • Development
  • Glutamatergic receptor
  • Maple syrup urine disease
  • Phosphorylation

ASJC Scopus subject areas

  • Developmental Neuroscience
  • Developmental Biology

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