title: Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction.
abstract: OBJECTIVE
  We investigated the relationship between butyrylcholinesterase (BuChE) activity and lipid oxidative damage in patients with disorders of propionate metabolism, before and after treatment with protein restriction and L-carnitine.
  
  
  METHODS
  BuChE activity and malondialdehyde (MDA) were measured in plasma from eight untreated patients (at diagnosis) and from seven patients under treatment with protein restriction and L-carnitne supplementation (100mg/kg/day).
  
  
  RESULTS
  We verified a significant reduction of butyrylcholinesterase activity, as well as an increased MDA formation in plasma from untreated patients. However, treated patients presented MDA and BuChE activity similar to controls. Furthermore, butyrylcholinesterase activity was negatively correlated with MDA concentrations in these patients.
  
  
  CONCLUSIONS
  The results suggest that an increased free radicals formation may be involved in the decrease of butyrylcholinesterase activity, possibly contributing to the neurological damage of these disorders, and that treatment with L-carnitine and low-protein diet possibly is able to prevent this damage.
authors.affiliation: Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Ipiranga 2752, Porto Alegre, RS, 90610-000, Brazil. grazischmitt@terra.com.br
authors.forename: Graziela S
authors.initials: GS
authors.lastname: Ribas
authors.forename: Emilene B
authors.initials: EB
authors.lastname: Scherer
authors.forename: Andrea G
authors.initials: AG
authors.lastname: Ferreira
authors.forename: Felipe
authors.initials: F
authors.lastname: Schmitz
authors.forename: Angela T
authors.initials: AT
authors.lastname: Wyse
authors.forename: Daiane
authors.initials: D
authors.lastname: Rodrigues
authors.forename: Sabrina
authors.initials: S
authors.lastname: Nascimento
authors.forename: Solange C
authors.initials: SC
authors.lastname: Garcia
authors.forename: Moacir
authors.initials: M
authors.lastname: Wajner
authors.forename: Carmen R
authors.initials: CR
authors.lastname: Vargas
mesh_terms: D002091:Butyrylcholinesterase / Q000097:blood* / Q000378:metabolism*; D002331:Carnitine / Q000737:chemistry / Q000494:pharmacology*; D002648:Child; D002675:Child, Preschool; D005609:Free Radicals; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015227:Lipid Peroxidation; D008055:Lipids / Q000737:chemistry; D008315:Malondialdehyde / Q000378:metabolism; D018384:Oxidative Stress; D011422:Propionates / Q000378:metabolism*; D011506:Proteins / Q000378:metabolism*
chemical_list: D005609:Free Radicals; D008055:Lipids; D011422:Propionates; D011506:Proteins; D008315:Malondialdehyde; D002091:Butyrylcholinesterase; D002331:Carnitine