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Related Experiment Video

Updated: Jul 21, 2026

Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

Published on: November 23, 2011

Metabolic and mitochondrial myopathies.

¹University of Oklahoma College of Medicine, Tulsa 74129-1077, USA.

Current Opinion in Rheumatology

|November 7, 1999

Summary

Genetic defects in energy metabolism cause metabolic myopathies, affecting individuals at any age. Advances in understanding these muscle diseases are leading to new treatments.

Area of Science:

Biochemistry
Genetics
Neurology

Background:

Metabolic myopathies stem from genetic defects impacting cellular energy production.
These rare genetic disorders manifest with diverse myopathic syndromes.
Clinical presentation varies, appearing at any life stage.

Purpose of the Study:

To summarize recent advancements in understanding metabolic myopathies.
To highlight the expansion of clinical descriptions and disease recognition.
To discuss the development of novel therapeutic strategies.

Main Methods:

Review of current scientific literature on metabolic myopathies.
Analysis of molecular and metabolic pathways involved in muscle energy disorders.
Synthesis of clinical data and therapeutic outcomes.

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Measurement of Mitochondrial Respiration in Human and Mouse Skeletal Muscle Fibers by High-Resolution Respirometry

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Last Updated: Jul 21, 2026

Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

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Published on: November 23, 2011

Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle

Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle

Published on: January 19, 2017

Measurement of Mitochondrial Respiration in Human and Mouse Skeletal Muscle Fibers by High-Resolution Respirometry

Measurement of Mitochondrial Respiration in Human and Mouse Skeletal Muscle Fibers by High-Resolution Respirometry

Published on: October 4, 2024

Main Results:

Significant progress in elucidating the molecular and metabolic underpinnings of these diseases.
Identification of previously unrecognized metabolic myopathy entities.
Emergence of innovative therapeutic interventions.

Conclusions:

Understanding the molecular basis of metabolic myopathies is crucial for diagnosis and treatment.
Continued research is expanding the spectrum of recognized metabolic myopathies.
New therapeutic avenues offer hope for improved patient outcomes.