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As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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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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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 dynamic abnormalities in amyotrophic lateral sclerosis.

Zhen Jiang1, Wenzhang Wang1, George Perry2

  • 1Department of Pathology, Case Western Reserve University, Cleveland, OH USA.

Translational Neurodegeneration
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PubMed
Summary

Amyotrophic lateral sclerosis (ALS) involves motor neuron loss, with mitochondrial dysfunction being an early sign. Targeting abnormal mitochondrial dynamics may offer a new therapeutic strategy for this motor neuron disease.

Keywords:
ALSMitochondrial biogenesis and mitophagyMitochondrial dynamicsMitochondrial dysfunctionMitochondrial fission and fusionMitochondrial trafficking

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Mitochondrial Biology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease with no cure.
  • Mitochondrial dysfunction is an early pathological hallmark of ALS.
  • Mitochondria are vital organelles with dynamic processes including fission, fusion, trafficking, and turnover.

Purpose of the Study:

  • To review mitochondrial dysfunction and dynamic abnormalities in ALS.
  • To explore targeting mitochondrial dynamics as a potential therapeutic strategy for ALS.

Main Methods:

  • Literature review of studies on mitochondrial dysfunction in ALS.
  • Analysis of research on mitochondrial dynamics in the context of ALS pathogenesis.

Main Results:

  • Abnormal mitochondrial dynamics are consistently reported in ALS.
  • Altered mitochondrial dynamics are implicated in the underlying mechanisms of mitochondrial dysfunction in ALS.

Conclusions:

  • Mitochondrial dysfunction and aberrant dynamics are key features of ALS.
  • Targeting mitochondrial dynamics presents a promising novel therapeutic avenue for ALS treatment.