scholarly journals Reduced mitochondrial DNA and OXPHOS protein content in skeletal muscle of children with cerebral palsy

Author(s):  
Ferdinand von Walden ◽  
Ivan J Vechetti ◽  
Davis Englund ◽  
Vandré C Figueiredo ◽  
Rodrigo Fernandez‐Gonzalo ◽  
...  
Author(s):  
Sudarshan Dayanidhi ◽  
Elisa H Buckner ◽  
Robin S Redmond ◽  
Henry G Chambers ◽  
Simon Schenk ◽  
...  

2019 ◽  
Vol 149 (10) ◽  
pp. 1863-1868
Author(s):  
Ibrahim Duran ◽  
Kyriakos Martakis ◽  
Mirko Rehberg ◽  
Christina Stark ◽  
Anne Koy ◽  
...  

ABSTRACT Background Densitometrically measured lean body mass (LBM) is often used to quantify skeletal muscle mass in children with cerebral palsy (CP). Since LBM depends on the individual's height, the evaluation of $\frac{{{\rm{LBM}}}}{{heigh{t^2}}}\ $ (lean BMI) is often recommended. However, LBM includes not only skeletal muscle mass but also the mass of skin, internal organs, tendons, and other components. This limitation applies to a far lesser extent to the appendicular lean mass index (LMIapp). Objectives The aim of the study was to evaluate skeletal muscle mass in children with CP using total lean BMI (LMItot) and LMIapp. Methods The present study was a monocentric retrospective analysis of prospectively collected data among children and adolescents with CP participating in a rehabilitation program. In total, 329 children with CP [148 females; Gross Motor Function Classification Scale (GMFCS) I, 32 children; GMFCS II, 73 children; GMFCS III, 133 children; GMFCS IV, 78 children; and GMFCS V, 13 children] were eligible for analysis. The mean age was 12.3 ± 2.75 y. Pediatric reference centiles for age-adjusted LMIapp were generated using data from NHANES 1999–2004. Low skeletal muscle mass was defined as a z score for DXA determined LMItot and LMIapp less than or equal to −2.0. Results The z scores for LMIapp were significantly lower than LMItot in children with CP, GMFCS levels II–V (P < 0.001), with the exception of GMFCS level I (P = 0.121), where no significant difference was found. The prevalence of low LMItot (16.1%; 95% CI: 16.1, 20.1%) was significantly lower (P < 0.001) than the prevalence of LMIapp (42.2%; 95% CI: 36.9, 47.9%) in the study population. Conclusions The prevalence of low skeletal muscle mass in children with CP might be underestimated by LMItot. LMIapp is more suitable for the evaluation of skeletal muscle mass in children with CP.


2020 ◽  
Vol 52 (7S) ◽  
pp. 295-296
Author(s):  
Sudarshan Dayanidhi ◽  
Alexia Rudofski ◽  
Marysol Encarnacíon ◽  
Jill Larson ◽  
Vineeta Swaroop

2009 ◽  
Vol 154 (5) ◽  
pp. 715-720.e1 ◽  
Author(s):  
David L. Johnson ◽  
Freeman Miller ◽  
Pravesh Subramanian ◽  
Christopher M. Modlesky

2021 ◽  
Vol 12 ◽  
Author(s):  
Sudarshan Dayanidhi

Skeletal muscle contractile proteins require a constant supply of energy to produce force needed for movement. Energy (ATP) is primarily produced by mitochondrial organelles, located within and around muscle fibers, by oxidative phosphorylation that couples electron flux through the electron transport chain to create a proton gradient across the inner mitochondrial membrane that is in turn used by the ATP synthase. Mitochondrial networks increase in size by biogenesis to increase mitochondrial abundance and activity in response to endurance exercise, while their function and content reduce with constant inactivity, such as during muscle atrophy. During healthy aging, there is an overall decline in mitochondrial activity and abundance, increase in mitochondrial DNA mutations, potential increase in oxidative stress, and reduction in overall muscular capacity. Many of these alterations can be attenuated by consistent endurance exercise. Children with cerebral palsy (CP) have significantly increased energetics of movement, reduced endurance capacity, and increased perceived effort. Recent work in leg muscles in ambulatory children with CP show a marked reduction in mitochondrial function. Arm muscles show that mitochondrial protein content and mitochondria DNA copy number are lower, suggesting a reduction in mitochondrial abundance, along with a reduction in markers for mitochondrial biogenesis. Gene expression networks are reduced for glycolytic and mitochondrial pathways and share similarities with gene networks with aging and chronic inactivity. Given the importance of mitochondria for energy production and changes with aging, future work needs to assess changes in mitochondria across the lifespan in people with CP and the effect of exercise on promoting metabolic health.


Sign in / Sign up

Export Citation Format

Share Document