Mitochondrial Dynamics: Molecular Mechanisms, Related Primary Mitochondrial Disorders and Therapeutic Approaches

Genes (Basel). 2021 Feb 10;12(2):247. doi: 10.3390/genes12020247.

Abstract

Mitochondria do not exist as individual entities in the cell-conversely, they constitute an interconnected community governed by the constant and opposite process of fission and fusion. The mitochondrial fission leads to the formation of smaller mitochondria, promoting the biogenesis of new organelles. On the other hand, following the fusion process, mitochondria appear as longer and interconnected tubules, which enhance the communication with other organelles. Both fission and fusion are carried out by a small number of highly conserved guanosine triphosphatase proteins and their interactors. Disruption of this equilibrium has been associated with several pathological conditions, ranging from cancer to neurodegeneration, and mutations in genes involved in mitochondrial fission and fusion have been reported to be the cause of a subset of neurogenetic disorders.

Keywords: mitochondrial diseases; mitochondrial dynamics; therapeutic approaches.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • ATPases Associated with Diverse Cellular Activities / genetics
  • ATPases Associated with Diverse Cellular Activities / metabolism
  • Dynamins / genetics
  • Dynamins / metabolism
  • GTP Phosphohydrolases / genetics
  • GTP Phosphohydrolases / metabolism
  • Gene Expression Regulation / drug effects*
  • Humans
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Metalloendopeptidases / genetics
  • Metalloendopeptidases / metabolism
  • Mitochondria / drug effects*
  • Mitochondria / metabolism
  • Mitochondria / pathology
  • Mitochondrial Diseases / drug therapy*
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / metabolism
  • Mitochondrial Diseases / pathology
  • Mitochondrial Dynamics / drug effects
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism
  • Neoplasms / drug therapy*
  • Neoplasms / genetics
  • Neoplasms / metabolism
  • Neoplasms / pathology
  • Neurodegenerative Diseases / drug therapy*
  • Neurodegenerative Diseases / genetics
  • Neurodegenerative Diseases / metabolism
  • Neurodegenerative Diseases / pathology
  • Organelle Biogenesis
  • Peptide Elongation Factors / genetics
  • Peptide Elongation Factors / metabolism
  • Protective Agents / therapeutic use*
  • Signal Transduction

Substances

  • MIEF2 protein, human
  • Membrane Proteins
  • Mff protein, human
  • Mitochondrial Proteins
  • Peptide Elongation Factors
  • Protective Agents
  • Metalloendopeptidases
  • YME1L1 protein, human
  • GTP Phosphohydrolases
  • MFN2 protein, human
  • OPA1 protein, human
  • ATPases Associated with Diverse Cellular Activities
  • DNM1L protein, human
  • Dynamins