Breakdown of adenine nucleotide pool in fatiguing skeletal muscle in McArdle's disease: a noninvasive 31P-MRS and EMG study

Muscle & Nerve
Jochen ZangeMatthias Vorgerd

Abstract

Energy metabolism and electrical muscle activity were studied in the calf muscles of 19 patients with proven McArdle's disease and in 25 healthy subjects. Phosphorus magnetic resonance spectroscopy and surface electromyography (S-EMG) were performed during two isometric muscle contractions of 3 min at 30% maximum voluntary contraction, one performed during normal perfusion and the other during applied ischemia. After about 1 min of ischemic muscle contraction in diseased muscle a significant acceleration in phosphocreatine breakdown was observed, along with a significant decrease in adenosine triphosphate. During both contractions the absence of glycolysis was shown by a significant alkalinization. Furthermore, in patients we observed a greater increase in the S-EMG amplitude than in control subjects. We conclude that early on during moderate exercise, a small number of muscle fibers reach metabolic depletion, indicated by a reduction in the adenine nucleotide pool. An increasing number of motor units, which are still in a high-energy state, are continuously recruited to compensate for muscle fatigue. This functional compartmentation may contribute to the pathophysiology of exercise intolerance in McArdle's disease.

References

Sep 1, 1992·The American Journal of Physiology·M J KushmerickT R Brown
May 1, 1990·International Journal of Sports Medicine·J M Lowenstein
Oct 1, 1990·Journal of Applied Physiology·K SahlinS F Lewis
Nov 1, 1987·Electroencephalography and Clinical Neurophysiology·T Masuda, T Sadoyama
Nov 1, 1987·Electroencephalography and Clinical Neurophysiology·T Sadoyama, T Masuda
Sep 1, 1986·Muscle & Nerve·J P BraakhekkeD F Stegeman
Dec 1, 1986·Brain : a Journal of Neurology·J P BraakhekkeE M Joosten
Dec 1, 1985·Journal of Applied Physiology·S F LewisR L Nunnally
Sep 1, 1984·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·D L ArnoldG K Radda
Dec 1, 1981·Klinische Wochenschrift·K W RumpfF Scheler
Nov 1, 1995·Journal of Applied Physiology·P Vestergaard-PoulsenO Henriksen
Mar 20, 1998·Annals of Neurology·M VorgerdM W Kilimann

❮ Previous
Next ❯

Citations

Aug 5, 2010·European Journal of Applied Physiology·Dale E RaeAlejandro Lucia
Oct 4, 2008·Nature Clinical Practice. Neurology·Alejandro LuciaJoaquín Arenas
May 21, 2010·Seminars in Musculoskeletal Radiology·Gregory ChangRavinder R Regatte
Jun 26, 2012·Brain : a Journal of Neurology·Gisela Nogales-GadeaAntoni L Andreu
Feb 22, 2012·PloS One·Gisela Nogales-GadeaAntoni L Andreu
Nov 25, 2006·Sports Medicine·Sébastien RatelCraig A Williams
Nov 16, 2013·European Journal of Applied Physiology·J KappensteinJ Zange
May 17, 2014·Biology·Yu Kitaoka
Jul 17, 2014·Sports Medicine·Alfredo SantallaAlejandro Lucia
Apr 11, 2006·Applied Physiology, Nutrition, and Metabolism = Physiologie Appliquée, Nutrition Et Métabolisme·Mark A Tarnopolsky
Feb 26, 2013·Molecular Genetics and Metabolism·Yu KitaokaMark A Tarnopolsky
Jul 27, 2010·PM & R : the Journal of Injury, Function, and Rehabilitation·Randall E Keyser
Nov 1, 2006·NMR in Biomedicine·Britton ChanceMartin Kushmerick
Jan 30, 2007·Journal of Magnetic Resonance Imaging : JMRI·Chris Boesch
Nov 13, 2015·Medicine and Science in Sports and Exercise·Gisela Nogales-GadeaAlejandro Lucia
Aug 3, 2013·Molecular Genetics and Metabolism·Yu KitaokaMark A Tarnopolsky
Apr 1, 2016·Medicine and Science in Sports and Exercise·Carmen Fiuza-LucesMaría Morán
Oct 16, 2015·Physiological Genomics·Gisela Nogales-GadeaAlejandro Lucia
Nov 2, 2021·Frontiers in Physiology·Eduardo Salazar-MartínezAlejandro Lucia

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.