Patients with mitochondrial myopathies (MM) usually suffer from exercise intolerance due to their impaired oxidative capacity and physical deconditioning. We evaluated the effects of a 12-week supervised randomized rehabilitation program involving endurance training in patients with MM. Twenty MM patients were assigned to a training or control group. For three nonconsecutive days each week, patients combined cycle exercise at 70% of their peak work rate with three upper-body weight-lifting exercises performed at 50% of maximum capacity. Training increased maximal oxygen uptake (28.5%), work output (15.5%), and minute ventilation (40%), endurance performance (62%), walking distance in shuttle walking test (+95 m), and peripheral muscle strength (32%-62%), and improved Nottingham Health Profile scores (21.47%) and clinical symptoms. Control MM patients did not change from baseline. Results show that our exercise program is an adequate training strategy for patients with mitochondrial myopathy.
Plasma carnitine "insufficiency," (plasma esterified carnitine to free carnitine ratio above 0.25) was found in 21 of 48 (43.8%) patients with mitochondrial myopathy, of whom 4 also showed both total and free carnitine deficiencies in plasma. In addition, plasma levels of SCAC and LCAC were higher in patients with mitochondrial myopathy than in controls (P < 0.001 and P < 0.01, respectively). Patients diagnosed as having plasma carnitine insufficiency or deficiency were treated with L-carnitine (50-200 mg/kg per day in four daily doses). Muscle weakness improved in 19 of 20 patients, failure to thrive in 4 of 8, encephalopathy in 1 of 9, and cardiomyopathy in 8 of 8 patients. Plasma carnitine "insufficiency" provides an additional clue to the diagnosis of mitochondrial myopathy and an indication for L-carnitine therapy.
In vitro protein digestibility (IVPD) of chickpea albumins and its possible relationship to their structure and the presence of trypsin inhibitor activity (TIA) have been studied. Trypsin digestion of the albumin fraction under non-reducing conditions was incomplete, while the reduction of interand intramolecular disulphide bonds caused an improvement in the accessibility of sites susceptible to trypsin digestion. Trypsin inhibitor activity in the chickpea albumin fraction was dependent upon both temperature and heating time. Although heating the albumin fraction at 100°C for 30 min reduced the TIA by more than 50% with respect to the initial activity, an important TIA rate was attributable to heat-resistant trypsin inhibitor. The TIA decrease was not related to an increase in the rate of IVPD. However, we observed a signi®cant (P 0.05) increment in IVPD in the presence of b-ME, con®rming the essential role of disulphide bonds in stabilising the protein structure of the albumin fraction.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.