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Mitochondria01:37

Mitochondria

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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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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.
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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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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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The Supercomplexes in the Crista Membrane01:41

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The mitochondrial cristae membrane is the primary site for the oxidative phosphorylation (OXPHOS) process of energy conversion mediated through respiratory complexes I to V. These complexes have been widely studied for decades, and it has been proven that they form supramolecular structures called respiratory supercomplexes (SC). These higher-order complexes may be crucial in maintaining the biochemical structure and improving the physiological activity of the individual complexes while...
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Mitochondrial Membranes01:45

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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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Mitochondria: Emerging Consequential in Sickle Cell Disease.

Mohammad S Akhter1, Hassan A Hamali1, Hina Rashid2

  • 1Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jizan 45142, Saudi Arabia.

Journal of Clinical Medicine
|February 11, 2023
PubMed
Summary

Mitochondria play a crucial role in sickle cell disease (SCD) pathophysiology, influencing cell fate and disease severity. Targeting mitochondrial dysfunction offers a promising therapeutic strategy for improving SCD outcomes.

Keywords:
ROSmitochondriamitochondrial retentionmtDNAsickle cell disease

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Area of Science:

  • Cell Biology
  • Genetics
  • Pathophysiology

Background:

  • Mitochondria are increasingly recognized for their roles beyond energy production, influencing cell structure, signaling, and fate.
  • Mitochondrial dysfunction is implicated in numerous genetic and acquired diseases.
  • Sickle cell disease (SCD) pathophysiology is increasingly linked to mitochondrial anomalies.

Purpose of the Study:

  • To review the multifaceted role of mitochondria in sickle cell disease.
  • To highlight the significance of mitochondrial dysfunction in SCD progression.
  • To explore therapeutic opportunities targeting mitochondria in SCD.

Main Methods:

  • Literature review of advanced mitochondrial multi-omics studies.
  • Analysis of research linking mitochondrial attributes to SCD pathology.
  • Synthesis of current understanding of mitochondrial involvement in SCD.

Main Results:

  • Mitochondrial aberrations, including altered mitophagy, increased reactive oxygen species (ROS), and mitochondrial DNA issues, contribute to SCD severity.
  • Mitochondrial dysfunction leads to red blood cell retention, increased cellular oxidation, inflammation, and worsened vaso-occlusive crises in SCD.
  • Emerging evidence underscores the critical role of mitochondria in SCD.

Conclusions:

  • Mitochondria are central to the pathological cascade of sickle cell disease.
  • Targeting specific impaired mitochondrial attributes presents a viable therapeutic avenue for SCD management.
  • Further research into mitochondria in SCD can lead to improved patient outcomes.