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Related Concept Videos

Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
Mitochondrial Precursor Proteins01:39

Mitochondrial Precursor Proteins

Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
Most of the mitochondrial precursors...
ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased ATP...
Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
Mitochondria01:37

Mitochondria

Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...

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Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
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Mitochondrial myopathies: developments in treatment.

Adam Hassani1, Rita Horvath, Patrick F Chinnery

  • 1Mitochondrial Research Group, Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, UK.

Current Opinion in Neurology
|July 24, 2010
PubMed
Summary
This summary is machine-generated.

Exercise training and Coenzyme Q10 supplementation show promise for mitochondrial myopathies. New strategies like PPAR/PGC-1alpha activation and ketogenic diets are also being investigated for these rare genetic muscle disorders.

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Last Updated: Jun 10, 2026

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
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Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle
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Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models

Published on: June 30, 2023

Area of Science:

  • Biochemistry
  • Genetics
  • Neurology

Background:

  • Mitochondrial myopathies are a group of rare genetic disorders affecting muscle function.
  • Limited effective treatment options exist, despite advances in understanding their molecular basis.

Purpose of the Study:

  • To review current and emerging treatment strategies for mitochondrial myopathies.
  • To highlight promising therapeutic avenues and ongoing research.

Main Methods:

  • Review of recent scientific literature on mitochondrial myopathy treatments.
  • Analysis of findings from preclinical studies and early clinical trials.

Main Results:

  • Exercise training demonstrates potential for improving exercise tolerance and oxidative capacity.
  • Coenzyme Q10 supplementation may benefit patients with Coenzyme Q10 deficiency, a common finding.
  • Activation of the peroxisome proliferator-activated receptor/peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PPAR/PGC-1alpha) pathway and ketogenic diets induce mitochondrial biogenesis in mouse models.

Conclusions:

  • Continued therapeutic trials for exercise and Coenzyme Q10 are recommended pending randomized clinical trial results.
  • Further research into PPAR/PGC-1alpha pathway activation, ketogenic diets, and other novel strategies is necessary for effective mitochondrial myopathy treatment.