Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction

• Published in: Clinical biochemistry 2012-01, Vol.45 (1-2), p.77-81
• Main Authors: Ribas, Graziela S., Scherer, Emilene B., Ferreira, Andrea G., Schmitz, Felipe, Wyse, Angela T., Rodrigues, Daiane, Nascimento, Sabrina, Garcia, Solange C., Wajner, Moacir, Vargas, Carmen R.
• Format: Article
• Language: English
• Subjects: Aminoacid disorders; Biological and medical sciences; Butyrylcholinesterase - blood; Butyrylcholinesterase - metabolism; Butyrylcholinesterase activity; Carnitine - chemistry; Carnitine - pharmacology; Child; Child, Preschool; Errors of metabolism; Free Radicals; Humans; Infant; Infant, Newborn; Investigative techniques, diagnostic techniques (general aspects); L-carnitine; Lipid Peroxidation; Lipids - chemistry; Malondialdehyde; Malondialdehyde - metabolism; Medical sciences; Metabolic diseases; Methylmalonic acidemia; Oxidative damage; Oxidative Stress; Propionates - metabolism; Propionic acidemia; Proteins - metabolism
• ISSN: 0009-9120; 1873-2933
• DOI: 10.1016/j.clinbiochem.2011.10.017

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. 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). 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. 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. ► Oxidative stress has been suggested in propionic and methylmalonic acidemias. ► Butyrylcholinesterase (BuChE) and malondialdehyde were measured in these patients. ► Malondialdehyde was increased, while BuChE was reduced in untreated patients. ► BuChE activity was negatively correlated with MDA levels. ► Treatment with L-carnitine and low-protein diet prevented these alterations.