Structure, function, and regulation of mitofusin-2 in health and disease

Gursimran Chandhok, Michael Lazarou, Brent Neumann

Research output: Contribution to journalArticleOtherpeer-review

31 Citations (Scopus)

Abstract

Mitochondria are highly dynamic organelles that constantly migrate, fuse, and divide to regulate their shape, size, number, and bioenergetic function. Mitofusins (Mfn1/2), optic atrophy 1 (OPA1), and dynamin-related protein 1 (Drp1), are key regulators of mitochondrial fusion and fission. Mutations in these molecules are associated with severe neurodegenerative and non-neurological diseases pointing to the importance of functional mitochondrial dynamics in normal cell physiology. In recent years, significant progress has been made in our understanding of mitochondrial dynamics, which has raised interest in defining the physiological roles of key regulators of fusion and fission and led to the identification of additional functions of Mfn2 in mitochondrial metabolism, cell signalling, and apoptosis. In this review, we summarize the current knowledge of the structural and functional properties of Mfn2 as well as its regulation in different tissues, and also discuss the consequences of aberrant Mfn2 expression.

Original languageEnglish
Pages (from-to)933-949
Number of pages17
JournalBiological Reviews
Volume93
Issue number2
DOIs
Publication statusPublished - 1 May 2018

Keywords

  • Charcot–Marie–Tooth disease
  • diabetes
  • mitochondria
  • mitochondrial dynamics
  • mitofusin-1
  • mitofusin-2
  • neurodegenerative disease
  • obesity
  • vascular disease

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