PMID- 38030066
OWN - NLM
STAT- Publisher
LR  - 20240103
IS  - 2213-2961 (Electronic)
IS  - 2213-2961 (Linking)
DP  - 2023 Nov 27
TI  - Dose-response effect of pre-exercise carbohydrates under muscle glycogen 
      unavailability: Insights from McArdle disease.
LID - S2095-2546(23)00115-1 [pii]
LID - 10.1016/j.jshs.2023.11.006 [doi]
AB  - BACKGROUND: This study aimed to determine the effect of different carbohydrate 
      (CHO) doses on exercise capacity in patients with McArdle disease-the paradigm of 
      "exercise intolerance", characterized by complete muscle glycogen 
      unavailability-and to determine whether higher exogenous glucose levels affect 
      metabolic responses at the McArdle muscle cell (in vitro) level. METHODS: 
      Patients with McArdle disease (n = 8) and healthy controls (n = 9) underwent a 
      12-min submaximal cycling constant-load bout followed by a maximal ramp test 15 
      min after ingesting a non-caloric placebo. In a randomized, double-blinded, 
      cross-over design, patients repeated the tests after consuming either 75 g or 150 
      g of CHO (glucose:fructose = 2:1). Cardiorespiratory, biochemical, perceptual, 
      and electromyographic (EMG) variables were assessed. Additionally, glucose uptake 
      and lactate appearance were studied in vitro in wild-type and McArdle mouse 
      myotubes cultured with increasing glucose concentrations (0.35, 1.00, 4.50, and 
      10.00 g/L). RESULTS: Compared with controls, patients showed the "classical" 
      second-wind phenomenon (after prior disproportionate tachycardia, myalgia, and 
      excess electromyographic activity during submaximal exercise, all p < 0.05) and 
      an impaired endurance exercise capacity (-51% ventilatory threshold and -55% peak 
      power output, both p < 0.001). Regardless of the CHO dose (p < 0.05 for both 
      doses compared with the placebo), CHO intake increased blood glucose and lactate 
      levels, decreased fat oxidation rates, and attenuated the second wind in the 
      patients. However, only the higher dose increased ventilatory threshold (+27%, 
      p = 0.010) and peak power output (+18%, p = 0.007). In vitro analyses revealed no 
      differences in lactate levels across glucose concentrations in wild-type 
      myotubes, whereas a dose-response effect was observed in McArdle myotubes. 
      CONCLUSION: CHO intake exerts beneficial effects on exercise capacity in McArdle 
      disease, a condition associated with total muscle glycogen unavailability. Some 
      of these benefits were dose dependent.
CI  - Copyright (c) 2024. Production and hosting by Elsevier B.V.
FAU - Valenzuela, Pedro L
AU  - Valenzuela PL
AD  - Physical Activity and Health Research Group ("PaHerg"), Research Institute of 
      Hospital "12 de Octubre" ("imas12"), Madrid 28041, Spain; Department of Systems 
      Biology, University of Alcala, Madrid 28871, Spain. Electronic address: 
      pedro.valenzuela92@gmail.com.
FAU - Santalla, Alfredo
AU  - Santalla A
AD  - Department of Sport and Computer Science, Section of Physical Education and 
      Sports, Faculty of Sport, Universidad Pablo de Olavide, Sevilla 41013, Spain; 
      EVOPRED Research Group, Universidad Europea de Canarias, Tenerife 38300, Spain.
FAU - Alejo, Lidia B
AU  - Alejo LB
AD  - Physical Activity and Health Research Group ("PaHerg"), Research Institute of 
      Hospital "12 de Octubre" ("imas12"), Madrid 28041, Spain; Universidad Europea de 
      Madrid, Faculty of Sport Sciences, Madrid 28670, Spain.
FAU - Merlo, Andrea
AU  - Merlo A
AD  - Gait & Motion Analysis Laboratory, Sol et Salus Hospital, Torre Pedrera di Rimini 
      (RN) 47922, Italy.
FAU - Bustos, Asuncion
AU  - Bustos A
AD  - Universidad Europea de Madrid, Faculty of Sport Sciences, Madrid 28670, Spain.
FAU - Castellote-Belles, Laura
AU  - Castellote-Belles L
AD  - Department of Clinical Biochemistry, Laboratoris Clinics, Hospital Universitari 
      Vall d'Hebron, Barcelona 08035, Spain.
FAU - Ferrer-Costa, Roser
AU  - Ferrer-Costa R
AD  - Department of Clinical Biochemistry, Laboratoris Clinics, Hospital Universitari 
      Vall d'Hebron, Barcelona 08035, Spain.
FAU - Maffiuletti, Nicola A
AU  - Maffiuletti NA
AD  - Human Performance Lab, Schulthess Clinic, Zurich 8008, Switzerland.
FAU - Barranco-Gil, David
AU  - Barranco-Gil D
AD  - Universidad Europea de Madrid, Faculty of Sport Sciences, Madrid 28670, Spain.
FAU - Pinos, Tomas
AU  - Pinos T
AD  - Biomedical Research Networking Center on Rare Disorders (CIBERER), Barcelona 
      08035, Spain; Mitochondrial and Neuromuscular Disorders Unit, Vall d'Hebron 
      Institut de Recerca, Universitat Autonoma de Barcelona, Barcelona 08035, Spain. 
      Electronic address: tomas.pinos@vhir.org.
FAU - Lucia, Alejandro
AU  - Lucia A
AD  - Physical Activity and Health Research Group ("PaHerg"), Research Institute of 
      Hospital "12 de Octubre" ("imas12"), Madrid 28041, Spain; Universidad Europea de 
      Madrid, Faculty of Sport Sciences, Madrid 28670, Spain.
LA  - eng
PT  - Journal Article
DEP - 20231127
PL  - China
TA  - J Sport Health Sci
JT  - Journal of sport and health science
JID - 101606001
SB  - IM
OTO - NOTNLM
OT  - Endurance
OT  - Glycogen storage disease
OT  - Glycogenosis
OT  - Nutrition
OT  - Supplement
EDAT- 2023/11/30 00:43
MHDA- 2023/11/30 00:43
CRDT- 2023/11/29 19:29
PHST- 2023/06/30 00:00 [received]
PHST- 2023/09/13 00:00 [revised]
PHST- 2023/10/20 00:00 [accepted]
PHST- 2023/11/30 00:43 [pubmed]
PHST- 2023/11/30 00:43 [medline]
PHST- 2023/11/29 19:29 [entrez]
AID - S2095-2546(23)00115-1 [pii]
AID - 10.1016/j.jshs.2023.11.006 [doi]
PST - aheadofprint
SO  - J Sport Health Sci. 2023 Nov 27:S2095-2546(23)00115-1. doi: 
      10.1016/j.jshs.2023.11.006.