Patients with organic acidaemias have an altered thiol status
Acta Pediatrica, 08/16/2012
Salmi H et al. – Patients with organic acidaemias may have a relative glutathione deficiency. With further research, these results could also have therapeutic implications.Methods
- In children with organic acidaemias, cysteine and glutathione concentrations in plasma and erythrocytes as well as erythrocyte glutathione peroxidase, glutathione reductase, glutathione S-transferase and glucose-6-phosphate dehydrogenase activities were studied.
- In addition, glutathione and cysteine concentrations were measured in human fibroblasts exposed to organic acids.
- Patients with organic acidaemias had lower plasma glutathione concentration than their controls.
- A greater fraction of glutathione and cysteine in the patients’ plasma was oxidised, suggesting decreased glutathione synthesis and increased consumption.