SciELO - Scientific Electronic Library Online

vol.24 issue3Use of sulfosalicylic acid in the detection of proteinuria and its application to hypertensive problems in pregnancyImmunopathogenesis of pemphigus vulgaris and pemphigus foliaceus author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand



Related links

  • On index processCited by Google
  • Have no similar articlesSimilars in SciELO
  • On index processSimilars in Google



Print version ISSN 0121-0793


OSORIO OROZCO, José Henry. Study of patients with type II glutaric aciduria by incubation of fibroblasts with tritiated palmitic and myristic acids. Iatreia [online]. 2011, vol.24, n.3, pp.267-271. ISSN 0121-0793.

Introduction: Glutaric aciduria type II (GA II), or multiple acyl-CoA dehydrogenase deficiency, is a disorder caused by deficiency of either electron transport flavoprotein or electron transport flavoprotein oxyreductase. It is an autsomal recessive metabolic disease, characterized by acidosis, hypoglycemia, organic aciduria, sweat-sock odour, and malformations in brain and kidneys. Objective: To analyse the oxidation rate of tritiated substrates by fibroblasts of patients with GA II. Materials and methods: Fibroblasts of two patients with GA II were incubated with tritiated palmitic and myristic acids. Results: Oxidation of tritiated substrates by fibroblasts of patients with GA II was very depressed (16%-18%) in comparison with controls. Conclusion: Diagnosis of GA II may be confirmed in vitro by the studied test.

Keywords : Fatty Acids; Glutaric aciduria type II; Metabolic disorders; Mitochondrial β -oxidation; Multiple dehydrogenase deficiency.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License