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Related Concept Videos

Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

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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...
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The present-day mitochondrial and chloroplast genomes have retained some of the characteristics of their ancestral prokaryotes and also have acquired new attributes during their evolution within eukaryotic cells. Like prokaryotic genomes, mitochondrial and chloroplast genomes neither bind with histone-like proteins nor show complex packaging into chromosome-like structures, as observed in eukaryotes. Unlike mitotic cell divisions observed in eukaryotic cells, mitochondria and chloroplasts...
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Export of Mitochondrial and Chloroplast Genes02:19

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A eukaryotic cell can have up to three different types of genetic systems: nuclear, mitochondrial, and chloroplast. During evolution, organelles have exported many genes to the nucleus; this transfer is still ongoing in some plant species. Approximately 18% of the Arabidopsis thaliana nuclear genome is thought to be derived from the chloroplast’s cyanobacterial ancestor, and around 75% of the yeast genome derived from the mitochondria’s bacterial ancestor. This export has occurred...
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Extraction: Advanced Methods00:56

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Metal ions can be separated from one another by complexation with organic ligands–the chelating agent– to form uncharged chelates. Here, the chelating agent must contain hydrophobic groups and behave as a weak acid, losing a proton to bind with the metal. Since most organic ligands used in this process are insoluble or undergo oxidation in the aqueous phase, the chelating agent is initially added to the organic phase and extracted into the aqueous phase. The metal-ligand complex is...
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Overview of Advanced Functional Groups02:22

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Functional groups are groups of atoms with specific chemical properties that occur within organic molecules and are sometimes denoted as “R”. Functional groups can “functionalize” a compound by enabling it to adopt different physical and chemical properties.
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The Inner Mitochondrial Membrane01:28

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The inner mitochondrial membrane is the primary site of ATP synthesis. The inner membrane domain that forms a smooth layer adjacent to the outer membrane is called the inner boundary membrane. This domain contains membrane transporters that drive metabolites in and out of the mitochondria.  In contrast, the inner membrane network that invaginates into the matrix space is called the cristae membrane. This domain accounts for principle mitochondrial function as it accommodates the protein...
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Updated: Jan 21, 2026

Author Spotlight: Advanced Integrated Model for Sepsis-Induced Myopathy and Single-Cell Metabolic Analysis
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Advances in primary mitochondrial myopathies.

Isabella Peixoto de Barcelos1, Valentina Emmanuele, Michio Hirano

  • 1Department of Neurology, Columbia University Irving Medical Center, New York, New York, USA.

Current Opinion in Neurology
|August 14, 2019
PubMed
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Emerging therapies show promise for mitochondrial myopathies, offering potential improvements beyond traditional treatments. Clinical trials for elamipretide and pyrimidine deoxynucleosides suggest enhanced patient outcomes and quality of life.

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Area of Science:

  • Mitochondrial Medicine
  • Translational Therapeutics
  • Neuromuscular Disorders

Background:

  • Mitochondrial diseases cause significant functional limitations.
  • Current treatments are largely supportive, including supplements and physical therapy.
  • Recent advances in translational medicine offer new therapeutic avenues.

Purpose of the Study:

  • To review recent findings in the treatment of mitochondrial myopathies.
  • To highlight emerging therapies showing promise in clinical trials.
  • To emphasize the need for further research and definitive diagnoses.

Main Methods:

  • Review of preliminary phase I and II clinical trials.
  • Analysis of compassionate-use data for specific therapies.
  • Comparison of outcomes with historical controls.

Main Results:

  • Elamipretide demonstrated safety and suggested improvements in the 6-minute walk test and fatigue scales in mitochondrial myopathies.
  • Pyrimidine deoxynucleosides showed preliminary safety and efficacy in survival and motor function for thymidine kinase 2-deficient myopathy.
  • These findings suggest potential for improved quality of life for patients.

Conclusions:

  • Emerging therapies like elamipretide and pyrimidine deoxynucleosides represent significant progress in treating mitochondrial myopathies.
  • Definitive diagnoses and natural history studies are crucial for understanding disease progression and evaluating new treatments.
  • Further research is essential to develop effective therapies that improve patient quality of life.