CMT 2A: Mitochondrial dynamics in Mammalian health and disease

[Guest guest]

Physiol Rev. 2009 Jul;89(3):799-845.

Mitochondrial dynamics in Mammalian health and disease.

Liesa M, Palacín M, Zorzano A.

The meaning of the word mitochondrion (from the Greek mitos, meaning thread, and

chondros, grain) illustrates that the heterogeneity of mitochondrial morphology

has been known since the first descriptions of this organelle. Such a

heterogeneous morphology is explained by the dynamic nature of mitochondria.

Mitochondrial dynamics is a concept that includes the movement of mitochondria

along the cytoskeleton, the regulation of mitochondrial architecture (morphology

and distribution), and connectivity mediated by tethering and fusion/fission

events.

The relevance of these events in mitochondrial and cell physiology has been

partially unraveled after the identification of the genes responsible for

mitochondrial fusion and fission.

Furthermore, during the last decade, it has been identified that mutations in

two mitochondrial fusion genes (MFN2 and OPA1) cause prevalent neurodegenerative

diseases (Charcot-Marie Tooth type 2A and Kjer disease/autosomal dominant optic

atrophy). In addition, other diseases such as type 2 diabetes or vascular

proliferative disorders show impaired MFN2 expression.

Altogether, these findings have established mitochondrial dynamics as a

consolidated area in cellular physiology. Here we review the most significant

findings in the field of mitochondrial dynamics in mammalian cells and their

implication in human pathologies.