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Related Concept Videos

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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Electron Transport Chain: Complex I and II01:46

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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...
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Animal Mitochondrial Genetics02:59

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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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Mitochondria01:37

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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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ATP Synthase: Mechanism01:48

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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...
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Translocation of Proteins into the Mitochondria01:19

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Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
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Author Spotlight: Decoding Mitochondrial Aging
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Author Spotlight: Decoding Mitochondrial Aging

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Mitochondrial diseases.

Maria J Molnar1, Gabor G Kovacs2

  • 1Institute of Genomic Medicine, Rare Disorders, Semmelweis University, Budapest, Hungary.

Handbook of Clinical Neurology
|October 9, 2017
PubMed
Summary
This summary is machine-generated.

Mitochondrial disorders, caused by mutations in nuclear or mitochondrial DNA, affect multiple organs. Advanced molecular genetics and sequencing have revolutionized their diagnosis.

Keywords:
mitochondrial DNAmitochondrial diseasenuclear DNAragged red fiberspongy vacuolation

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Area of Science:

  • Medicine
  • Genetics
  • Neurology

Background:

  • Mitochondrial disorders pose significant medical challenges.
  • They stem from mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA).
  • These disorders often impact multiple organs but can be organ-specific.

Purpose of the Study:

  • To summarize the diagnostic approaches for mitochondrial disorders.
  • To highlight neuropathologic findings in affected individuals.
  • To underscore the impact of recent advancements on diagnosis.

Main Methods:

  • Diagnosis involves clinical, biochemical, histopathologic, functional, and molecular genetic assessments.
  • Neuropathologic examination reveals characteristic alterations in muscle and brain tissue.
  • Molecular genetics, including exome and whole-genome sequencing, are key diagnostic tools.

Main Results:

  • Muscle pathology varies from ragged red fibers to minimal changes.
  • Brain pathology commonly includes white and gray matter vacuolation, neurodegeneration, and vascular changes.
  • New sequencing technologies have transformed diagnostic strategies.

Conclusions:

  • Mitochondrial disorders require a multi-faceted diagnostic approach.
  • Understanding neuropathologic features aids in diagnosis and research.
  • Molecular genetics and advanced sequencing are pivotal for accurate and efficient diagnosis of mitochondrial diseases.