Lactate disposal via gluconeogenesis is increased during exercise in patients with mitochondrial myopathy due to complex I deficiency
- Resource Type
- Authors
- Dirk-Jan Reijngoud; Mark J. Roef; Ruud Berger; Satish C. Kalhan; Kees de Meer
- Source
- University of Groningen
Pediatric Research, 51(5), 592-597. Nature Publishing Group
Scopus-Elsevier
- Subject
- Adult
Blood Glucose
Male
medicine.medical_specialty
Adolescent
Physical Exertion
Physical exercise
Fatty Acids, Nonesterified
Mitochondrion
Biology
GLUCOSE
chemistry.chemical_compound
Oxygen Consumption
Mitochondrial myopathy
Internal medicine
ETHANOL
medicine
Humans
NADH, NADPH Oxidoreductases
Child
Pyruvates
Triglycerides
Electron Transport Complex I
Triglyceride
Heparin
fungi
Gluconeogenesis
Mitochondrial Myopathies
Fasting
Deuterium
medicine.disease
Pathophysiology
Lactic acid
FASTED STATE
Endocrinology
chemistry
Child, Preschool
Pediatrics, Perinatology and Child Health
Exercise Test
Lactates
Female
TURNOVER
Energy Metabolism
medicine.drug
- Language
- ISSN
- 0031-3998
This study evaluated lactate disposal via gluconeogenesis as well as effects of FFA availability on gluconeogenesis via pyruvate (GNG(PYR)) in patients with mitochondrial myopathy due to complex I deficiency (CID). The rates of GNG(PYR) were measured in three CID patients and six healthy controls at rest and during 90 min cycle exercise, using the deuterium-labeled water method. All subjects served as their own control: on one occasion they were studied in the fasting state, and on the second occasion they received an infusion of triacylglycerol plus heparin, At rest. the fractional rate of gluconeogenesis from pyruvate was higher in patients than in controls in the fasting state. Triacylglycerol infusion was associated with increased rates of GNG(PYR) at rest in controls (p