Duchenne muscular dystrophy: a model for studying the contribution of muscle to energy and protein metabolism

Hankard, R.

doi:10.1051/rnd:19980205

Cited by 8 publications

(7 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These data are consistent with previous reports of high FSRs in muscles of mdx mice [36] and DMD boys [62], [63], although this has not been a consistent finding in humans [64], [65]. The change in gastrocnemius protein FSR with age in mdx mice can be attributed to both the decrease that occurs with muscle maturation in intact regenerated myofibers [66], and also the reduced proportion of myofibers that are hypertrophying and turning over their proteins more rapidly in the early phase of regeneration.…”

Section: Discussionsupporting

confidence: 90%

Dystropathology Increases Energy Expenditure and Protein Turnover in the Mdx Mouse Model of Duchenne Muscular Dystrophy

et al. 2014

View full text Add to dashboard Cite

The skeletal muscles in Duchenne muscular dystrophy and the mdx mouse model lack functional dystrophin and undergo repeated bouts of necrosis, regeneration, and growth. These processes have a high metabolic cost. However, the consequences for whole body energy and protein metabolism, and on the dietary requirements for these macronutrients at different stages of the disease, are not well-understood. This study used juvenile (4- to 5- wk-old) and adult (12- to 14-wk-old) male dystrophic C57BL/10ScSn-mdx/J and age-matched C57BL/10ScSn/J control male mice to measure total and resting energy expenditure, food intake, spontaneous activity, body composition, whole body protein turnover, and muscle protein synthesis rates. In juvenile mdx mice that have extensive muscle damage, energy expenditure, muscle protein synthesis, and whole body protein turnover rates were higher than in age-matched controls. Adaptations in food intake and decreased activity were insufficient to meet the increased energy and protein needs of juvenile mdx mice and resulted in stunted growth. In (non-growing) adult mdx mice with less severe dystropathology, energy expenditure, muscle protein synthesis, and whole body protein turnover rates were also higher than in age-matched controls. Food intake was sufficient to meet their protein and energy needs, but insufficient to result in fat deposition. These data show that dystropathology impacts the protein and energy needs of mdx mice and that tailored dietary interventions are necessary to redress this imbalance. If not met, the resultant imbalance blunts growth, and may limit the benefits of therapies designed to protect and repair dystrophic muscles.

show abstract

Section: Discussionsupporting

confidence: 90%

Dystropathology Increases Energy Expenditure and Protein Turnover in the Mdx Mouse Model of Duchenne Muscular Dystrophy

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Unpaired Student's t-test was used to compare mean differences in body composition and energy expenditure between obese and non-obese Duchenne children and between patients and healthy children of the same age. The values of body composition and energy expenditure of healthy children were obtained from the literature (Leroy- Willig et al, 1997;Palmieri et al, 1996;Hankard, 1998, Maffeis et al, 1991Weinsier et al, 1992). Significance was established at P < 0.05.…”

Section: Discussionmentioning

confidence: 99%

Body composition and energy expenditure in Duchenne muscular dystrophy

et al. 2003

View full text Add to dashboard Cite

Objective: To investigate the relationship between resting energy expenditure (REE) and body composition in Duchenne Muscular Dystrophy (DMD). Design: An observational study. Setting: University Research Centre. Subjects: Nine Duchenne children (age range 6 -12 y), mean relative weight 128%, agreed to undergo the investigation and all of them completed the study; Interventions: Assessment of body composition (total body fat and skeletal muscle mass) by magnetic resonance imaging and resting energy expenditure by indirect calorimetry. Main outcome measures: Fat mass (FM; kg and percentage weight), fat-free mass (FFM; kg and percentage weight), muscle mass (kg and percentage weight), resting energy expenditure (kJ=kg body weight and kJ=kg fat-free mass). Results: In Duchenne children fat mass averages 32% and total skeletal muscle mass 20% of body weight. Resting energy expenditure per kg of body weight falls within the normal range for children of the same age range, while when expressed per kg of FFM is significantly higher than reference values. No relationship was found between REE and total skeletal muscle mass. Conclusions:Our results do not demonstrate a low REE in DMD boys; on the contrary REE per kg of FFM is higher than normal, probably due to the altered FFM composition. We suggest that the development of obesity in DMD children is not primarily due to a low REE but to other causes such as a reduction in physical activity and or overfeeding.

show abstract

“…Furthermore, tissue heterogeneity can complicate the analysis of these disorders. For example, whereas myofibrillar damage reduces the number of functioning muscle fibers in Duchenne muscular dystrophy, the mass of the muscle often actually increases due to the infiltration of fat and connective tissue (14,17,23,52).…”

Section: Discussionmentioning

confidence: 99%

“…Atrophy and subsequent muscle weakening accompanies the aging process in both humans and animal models (26). Disuse atrophy is a significant consequence in a wide range of disorders that result in immobility, including spinal cord injury, unloading, weightlessness, chronic glucocorticoid treatment, sepsis, and muscular dystrophies (8,10,14,17,21,23,25,42,43,52). In some cases subsequent organismal death results from respiratory arrest (3).…”

mentioning

confidence: 99%

Changes in contractile properties of skeletal muscle during developmentally programmed atrophy and death

Schwartz

Ruff

2002

American Journal of Physiology-Cell Physiology

View full text Add to dashboard Cite

Schwartz, Lawrence M., and Robert L. Ruff. Changes in contractile properties of skeletal muscle during developmentally programmed atrophy and death. Am J Physiol Cell Physiol 282: C1270-C1277, 2002. First published January 16, 2002 10.1152/ajpcell.01275.2000.-Skeletal muscle atrophy and death are protracted processes that accompany aging and pathological insults in mammals. The intersegmental muscles (ISMs) from the tobacco hawkmoth Manduca sexta are composed of giant fibers that undergo distinct hormonally-regulated programs of atrophy and death at the end of metamorphosis. Atrophy occurs during the 3 days preceding adult emergence and results in a 40% reduction of mass, whereas death takes place during the subsequent 30 h and results in the complete loss of the fibers. There are no significant changes in tetanic force or calcium sensitivity in skinned fiber preparations during atrophy. However, the size of caffeine-induced contractions fell by about 50%. With the onset of the death phase, dramatic reductions occur in ISM: tetanic force, twitch amplitude, resting potential, caffeineinduced contractions, calcium sensitivity, and Hill coefficients. Several lines of evidence suggest that ISM atrophy is caused by an increase in protein turnover without significant modification of fiber organization. In contrast, ISM death is accompanied by disorganization of the contractile apparatus and concomitant loss of contractile function.

show abstract

Duchenne muscular dystrophy: a model for studying the contribution of muscle to energy and protein metabolism

Abstract: -Duchenne muscular dystrophy (DMD) is associated with a dramatic muscle mass loss. We hypothesized that DMD would be associated with significant changes in both energy and protein metabolism. We studied the resting energy expenditure (REE)

Cited by 8 publications

References 15 publications

Dystropathology Increases Energy Expenditure and Protein Turnover in the Mdx Mouse Model of Duchenne Muscular Dystrophy

Dystropathology Increases Energy Expenditure and Protein Turnover in the Mdx Mouse Model of Duchenne Muscular Dystrophy

Body composition and energy expenditure in Duchenne muscular dystrophy

Changes in contractile properties of skeletal muscle during developmentally programmed atrophy and death

Contact Info

Product

Resources

About