M Tarnopolsky

..Increased awareness of these disorders by exercise physiologists and sports medicine practitioners should lead to more accurate and more rapid diagnosis and the opportunity for therapy and genetic counseling...

..05). Menstrual cycle had a small effect on PPARdelta, GP, and glycogenin mRNA content. Overall, women have greater mRNA content for several genes involved in lipid metabolism, which is partially due to an effect of E(2)...

..This article focuses on the usefulness of exercise in the evaluation of genetic metabolic myopathies...

..In addition, women have higher IMCL content compared with men due mainly to a greater number of individual droplets. Finally, endurance exercise training increased the proportion of IMCL in physical contact with mitochondria...

..The purpose of this paper is to review the pharmacology and potential mechanism(s) of action of caffeine and creatine as they pertain to possible use as an ergogenic aid in sport...

..6 gPRO/kg/d. Although most endurance athletes get enough protein to support any increased requirements, those with low energy or carbohydrate intakes may require nutritional advice to optimize dietary protein intake...

..To determine whether creatine monohydrate (CrM) supplementation increases strength and fat-free mass (FFM) in boys with Duchenne muscular dystrophy (DD)...

..Preliminary data strongly suggest that mitochondrial DNA shifting occurs in skeletal muscle from older adults following resistance-exercise training...

..The results suggest that the development of exercise-induced paracrystalline inclusions may be influenced by the G15497A mtDNA mutation, and that CM mitigates against the pathological consequences of this mutation...

..SCO2 mutations should be screened in suspected SMA cases with normal smn mutation analysis and any one of; cardiomyopathy, lactic acidosis, or COX deficiency in muscle...

..The male patient in the second pedigree showed features of optic neuropathy without spasticity/dystonia. These results further support that the 14,459 G --> A transition mutation is causally related to LHON and spasticity/dystonia...

..In summary, the ergogenic effects of caffeine during endurance activity are mediated partly by enhanced contractile force and partly by a reduction in perceived exertion, possibly though a blunting of effort and (or) pain...

....

..To evaluate the evidence for dietary recommendations in older adult athletes...

..Trial Registration. ClinicalTrials.gov NCT00473902...

..50-fold. None of the mRNA species, or muscle fiber type, size, or strength, was different between legs. These findings are important for the design and analysis of studies using muscle data in older adults...

..In summary, metabolic myopathies provide important insights into regulatory and nutritional aspects of the major biochemical pathways of intermediary metabolism in human skeletal muscle...

..The greater fat oxidation for women during submaximal endurance exercise compared with men seems to occur partly through a sex hormone-mediated enhancement of lipid-oxidation pathways...

..It will also be important to determine whether these changes are maintained over a longer time period...

....

..e., corticosteroids)...

..Bergström needle muscle biopsies have been used by exercise physiologists for over 35 years but have been less accepted by neuromuscular clinicians due to size concerns...

..Most patients with metabolic myopathies can participate in sports with appropriate exercise adaptations and dietary manipulation...

..5 mmol . kg dry muscle-1 . h-1; P < 0.001) with no gender differences. We conclude that postexercise CHO and CHO-Pro-Fat nutritional supplements can increase glycogen resynthesis to a greater extent than Pl for both men and women...

..We have recently shown that timing of nutrient delivery in the postexercise period can have positive effects on whole body protein turnover (B. D. Roy et al., Med Sci Sports Exerc. 32(8):1412-1418, 2000)...

..4. In summary, complete and partial muscle AMPD deficiency did not affect TCA cycle anaplerosis, phosphocreatine hydrolysis, energy charge or exercise performance...

..05). There were no gender differences in the proportion of pro- and macroglycogen or hexokinase activity. A low energy intake may explain the previously reported lower capacity for women to glycogen load compared with men...

..This further supports the belief that creatine monohydrate supplementation may benefit patients with low muscle creatine stores, although the reduction in creatine transporter protein may have implications for dosing...

..These data support the hypothesis that some of the ergogenic effect of caffeine in endurance exercise performance occurs directly at the skeletal muscle level...

..Creatine administration increased all measured indices in both studies. Short-term creatine monohydrate increased high-intensity strength significantly in patients with neuromuscular disease...

....

..05). These results demonstrated that Cr-mH supplementation during weight training resulted in increases in skeletal muscle total creatine without reductions in CRT protein and acute Cr-mH loading did not decrease CRT mRNA content...

..Species differences must be considered in the evaluation of toxicological and physiological studies...

..Thus, a modified FIT was helpful in the diagnosis of GSD and excluding AMPD deficiency, but not in the diagnosis of MITO disease. Catheter placement is critical to the interpretation of a FIT, whereas pretesting diet is less important...

....

..In conclusion, unilateral resistance exercise training is effective in enhancing the skeletal muscle cellular antioxidant capacity in older adults. The potential long-term benefits of these adaptations remain to be evaluated...

..The results also suggest that increases in SIRT1, nuclear PGC-1alpha, and Tfam may be involved in coordinating mitochondrial adaptations in response to HIT in human skeletal muscle...

..We conclude that resistance exercise potentiates the effect of feeding on the phosphorylation and presumably activation of critical proteins involved in the regulation of muscle protein synthesis in young men...

..These factors likely contribute to age-related reductions in mitochondrial function and lipid metabolism...

..In addition, BWST training can bring about improvements in ambulatory capacity and antiatherogenic changes in blood lipid profile...

..05). We conclude that 1). the contraction-induced increase in flux through AAT is reduced after 5 days of aerobic training and 2). the muscle glycogenolytic response during exercise after STT in women is similar to that in men...

..This article gives an overview of these different impacts on endurance exercise, concluding with an examination of the metabolic myopathies that impair substrate metabolism in skeletal muscle and result in exercise intolerance...

....

..Importantly, post-exercise supplementation improved time to exhaustion during a subsequent bout of endurance exercise...

..The addition of CrM supplementation to the exercise stimulus enhanced the increase in total and fat-free mass, and gains in several indices of isometric muscle strength...

..CRF2R-treated mice had decreased heart weights compared to placebo-treated mice. We conclude that CRF2R agonists should be further evaluated as a potential therapy for dystrophinopathies...

..Acute consumption of fat-free fluid milk after resistance exercise promotes a greater positive protein balance than does soy protein...

..Endurance exercise resulted in a net decrease in IMCL density due to decreased number of IMCL, not decreased IMCL size, in both sexes...

..Furthermore, antioxidant therapy might prove helpful in the management of this disease...

....

..02) in all subjects and CuZnSOD protein content (P=0.04) in obese women, in the absence of changes in body weight or composition. ET without weight loss decreases systemic oxidative stress, but not markers of inflammation, in obese women...

..05). In conclusion, E(2) might partially regulate lipid metabolism in skeletal muscle by altering the protein content of MCAD, which may be directly or indirectly regulated by an increase in PGC-1alpha and reduction in miR-29b...

..02) and FOS at 48H (P<0.05). These findings indicate that early RhoA and NFAT signaling and regulation are altered following exercise for muscle remodeling and repair, but are not affected by E2...

..05). Sixty days of moderate- to high-dose CoQ(10) treatment had minor effects on cycle exercise aerobic capacity and post-exercise lactate but did not affect other clinically relevant variables such as strength or resting lactate...

..Furthermore, we have described an effective and safe knee immobilization method that results in reductions in quadriceps muscle strength and size...

..01), and smaller retroperitoneal fat pad stores (P < 0.001). This study provided evidence for therapeutic benefit from a four-compound combination therapy alone, and in conjunction with corticosteroids in the mdx model of DMD...

..5-fold, respectively). Taken together, our data demonstrate oxidative stress and compensatory antioxidant enzyme upregulation in SOD1-G93A skeletal muscle...

..001) and a greater PG utilization (P = 0.05) compared with LP women. We conclude that sex, and to a lesser extent menstrual cycle, influence glucose turnover and glycogen utilization during moderate-intensity endurance exercise...

..These results demonstrate a compartmentalized pattern of subcellular G deposition in human skeletal muscle for both the size and density of granules...

..The offspring of nicotine-exposed mothers also had elevated blood pressure. These data demonstrate that adverse effects of fetal and neonatal exposure to nicotine can influence aspects of metabolic risk in subsequent generations...

..0001), but exhibited poor agreement (mean bias +/- 1SD = -1.4 +/- 2.1 pg/mL). The CBA provided concordant IL-6 concentrations at rest, when detected, but underestimated exercise-induced increases in IL-6 versus the ELISA method...

....

....

..In conclusion, healthy aging is associated with oxidative damage to proteins and DNA, a compensatory up-regulation of antioxidant enzymes, and aberrations of mtDNA, with no reduction in electron transport chain maximal enzyme activity...

..Increases in the capacity for transport and phosphorylation glucose in skeletal muscle likely play a role in these adaptations...

..Until this time, an accurate diagnosis will facilitate treatment on a case-by-case basis...

..01) but not myotonic dystrophy, as compared to healthy controls. These results indicate that 8-OHdG excretion can be used as a marker of oxidative stress in clinical trials with dystrophinopathy...

..The purpose of this study was to investigate the expression levels of LCAD in human skeletal muscle...

..8-fold higher serum vitamin E concentration (P < 0.01), had no affect on indices of contraction-induced muscle damage nor inflammation (macrophage infiltration) as a result of eccentrically biased muscle contractions...

..405, P = 0.003). Short-term endurance training without weight loss does not alter hepatic lipid content. There was a strong relationship between GGT/ALT and body composition (percent BF) as well as between ALT and hepatic lipid content...

..Moreover, increases in complex IV of the electron transport chain may have an indirect antioxidant effect in older adults and may improve function in daily activities...

....

....

..These results demonstrate sex-based differences in the distribution of NK cell subsets and activation status in response to exercise, but not CHO intake, and further support the need to control for sex in exercise immunology studies...

..Catalase, Bcl-2 and caspases did not differ. We conclude that CR increases lipid peroxidation, inflammation and apoptosis, while decreasing mitochondrial bioenergetic efficiency, protein oxidation and stress response in G93A mice...

..We postulate that an immune-mediated disruption of caveolar function was exacerbated by statin exposure. We are unaware of any previous cases of statin-mediated unmasking of RMD...

..These results are of relevance for therapeutic exercise in patients with dystrophinopathy where exercise prescription remains controversial...

..05). Given the large difference in training volume, these data demonstrate that SIT is a time-efficient strategy to induce rapid adaptations in skeletal muscle and exercise performance that are comparable to ET in young active men...

....

..In conclusion, women have more protein content of the major enzymes involved in long and medium chain fatty acid oxidation which could account for the observed differences in fat oxidation during exercise...

..001). In conclusion, some of the reduction in muscle performance in older adults may be due to lower activity of the anaerobic and aerobic enzymes as well as protein content, not solely due to a decrease in physical activity...

..In particular, they suggest that metabolic processes in the hippocampus may have an important role in the neurobiology of anxiety...

..The anabolic effect of resistance exercise is enhanced by the provision of dietary protein...

..Further research is needed to find if the SEPCs detected in our patient is an association or a coincidental finding...

..This suggests that E2 attenuates neutrophil infiltration; however, the mechanism does not appear to be lesser oxidative stress or membrane damage and may indicate lesser neutrophil/endothelial interaction...

..In conclusion, the reduction in desmin disruption and Z-band streaming indicates that CCB attenuated, or delayed, the contraction-induced damage to sarcomeric proteins...

..kg FFM(-1)) in the control condition. A nutritive effect of post-exercise protein content was not discernible short term (15 h), but a delayed performance benefit (60 h) was observed following protein-enriched high-carbohydrate ingestion...

....

..05); however, there was no effect of gender on percent fatigue. CONCLUSIONS: Future studies must consider sampling times after metabolic stress in order to quantify changes in MDA concentration...

..These findings indicate that co-ingestion of alpha-lipoic acid with creatine and a small amount of sucrose can enhance muscle total creatine content as compared to the ingestion of creatine and sucrose or creatine alone...

..Based on these observations, ingested carbohydrate may be a particularly beneficial source of fuel during endurance exercise for females...

..2 microm vs. 55.4 +/- 14.8 microm), strength, and activities of daily living (ADL) times. Thus, patients respond to resistance training with muscle fiber adaptations, and improvements in strength and function. Creatine was not beneficial...

..These results support the hypothesis that a reduced oxidative capacity, due in part to age-related mitochondrial dysfunction, contributes to the decline in aerobic performance in aging skeletal muscles...

..copper and water-soluble vitamins). These data indicate that a substantial number of adults with muscular dystrophy do not meet current dietary intake recommendations. The potential clinical implications of these findings are discussed...

..Furthermore, E2 reduces the basal level of total muscle glycogen, particularly the proglycogen form...

..Assuming we can extrapolate these results to humans, short-term CR should be avoided in patients with amyotrophic lateral sclerosis, especially men...

..Future studies with larger sample sizes in relatively homogeneous groups will be required to determine whether such combination therapies influence function and quality of life...

..Proteins from different sources may differ in their ability to support muscle protein accretion because of different patterns of postprandial hyperaminoacidemia...

..We are the first to report this large-scale gene expression in the skeletal muscle with short-term CrM supplementation, a response that suggests changes in cellular osmolarity...