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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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Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes02:16

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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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The Inner Mitochondrial Membrane01:28

The Inner Mitochondrial Membrane

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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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Mitochondrial Membranes01:45

Mitochondrial Membranes

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A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
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Mitochondrial Protein Sorting01:39

Mitochondrial Protein Sorting

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Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
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Author Spotlight: Establishing a New Fluorescence-Based Protocol for In Vivo Mitochondrial Morphology Analysis in Parkinson's Disease
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Mitochondrial diseases.

Ryan L Davis1, Christina Liang2, Carolyn M Sue2

  • 1Department of Neurogenetics, Kolling Institute of Medical Research, Royal North Shore Hospital and University of Sydney, Sydney, NSW, Australia.

Handbook of Clinical Neurology
|January 13, 2018
PubMed
Summary
This summary is machine-generated.

Mitochondrial diseases are common inherited metabolic disorders affecting cellular energy. Advances in testing and treatments offer hope for better diagnosis and management of these complex conditions.

Keywords:
FGF-21GDF-15biomarkersinherited metabolic diseasemitochondrial diseasemtDNA

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Area of Science:

  • Biochemistry
  • Genetics
  • Cell Biology

Background:

  • Mitochondrial diseases are inherited disorders impacting cellular energy production.
  • They are the most frequent inherited metabolic diseases, affecting all ages with varied symptoms.
  • Mitochondrial dysfunction can impact multiple organ systems, complicating diagnosis.

Purpose of the Study:

  • To review the current understanding of mitochondrial diseases.
  • To highlight diagnostic challenges and recent advancements.
  • To discuss emerging treatments and reproductive options.

Main Methods:

  • Literature review of recent research on mitochondrial diseases.
  • Analysis of diagnostic tools, including biomarker and genetic testing.
  • Examination of current and future therapeutic strategies.

Main Results:

  • Mitochondrial diseases are diverse, affecting energy production and multiple organ systems.
  • Diagnosis is challenging due to variable presentations.
  • New biomarkers, genetic tests, and treatments are improving patient care.

Conclusions:

  • Recent progress in diagnostics and therapeutics significantly enhances the management of mitochondrial diseases.
  • These advancements offer improved identification and clinical outcomes for patients.
  • Further research holds promise for addressing the complexities of these disorders.