BACKGROUND Diagnosis of mitochondrial disorders usually requires a muscle biopsy to examine mitochondrial function. We describe our diagnostic procedure and results for 29 patients with mitochondrial disorders. METHODS Muscle biopsies were from 43 healthy individuals and 29 patients with defects in one of the oxidative phosphorylation (OXPHOS) complexes, the pyruvate dehydrogenase complex (PD...

At rest, administration of the short-chain fatty acid acetate suppresses fat oxidation without affecting carbohydrate utilization. The combined effect of increased acetate availability and exercise on substrate utilization is, however, unclear. With local ethics approval, we studied the effect of ingesting either sodium acetate (NaAc) or sodium bicarbonate (NaHCO3) at a dose of 4 mmol·kg-1 body...

the aim of the present study was to examine the effect of menstrual cycle on substrate metabolism, energy expenditure and performance during exhaustive incremental exercise in female students. for this purpose, 15 female students (mean age 21.17 ± 1.47 years, bmi 20.55 ± 1.71 kg/m2) participated voluntarily in this study. the design was adjusted in three stages: bleeding, follicular and the lat...

Saturation mutagenesis was performed over six residues delimiting the substrate binding pocket of a fungal laccase previously engineered in the lab. Mutant libraries were screened using sinapic acid as a model substrate, and those mutants presenting increased activity were selected for exploring the oxidation of lignin-derived phenols. The latter comprised a battery of phenolic compounds of int...

fat oxidation during exercise can be influenced by exercise intensity and duration, training status, diet and physical activity level. however, few studies have investigated the effect of time of day on the rate of fat oxidation during exercise. thus the present study was designed to investigate the effect of time of day on indexes of fat oxidation in active young women. for this purpose, 14 he...

the present study aimed to compare substrate oxidation and total energy expenditure during running with low and moderate intensity until exhaustion and one hour after exercise in obese male students. for this purpose, in a crossover study design, 11 male university students (mean age 20.45±1.69 years, bmi 31.26±1.3 kg/m2, body fat percentage 28.47±3.53 %) participated in this study. two exercis...

Understanding the molecular determinants of enzyme performance is of primary importance for the rational design of ad hoc mutants. A novel approach, which combines efficient conformational sampling and quick reactivity scoring, is used here to shed light on how substrate oxidation was improved during the directed evolution experiment of a fungal laccase (from Pycnoporus cinnabarinus), an indust...

Oxy-ferrous substrate-bound cytochrome P-450cam (mrsO2) autooxidizes in the absence of its specific effector protein, putidaredoxin, without hydroxylating the substrate, camphor. The autooxidation is first order with an activation energy of 17 kcal mol-1 at 25 degrees, pH 7.0. Substrate removal and low pH accelerate the reaction. The product, 5-exo-OH camphor, and a nonhydroxylated pseudosubstr...