Get the balance right: Mitofusins roles in health and disease CMT2A

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Biochim Biophys Acta. 2006 Mar 9

Get the balance right: Mitofusins roles in health and disease.

Santel A.

Atugen AG/SR Pharma plc, Otto-Warburg-Haus, -Rossle-Str.10,

13125 Berlin, Germany.

Mitochondria are highly dynamic organelles exhibiting an elaborate

morphology and fine structure. Fusion and fission processes

contribute to the maintenance and dynamics of mitochondrial

morphology. The Mitofusins, a class of evolutionary conserved GTPases

of the mitochondrial outer membrane, are essential for the controlled

fusion of mitochondrial membranes. Genetic and biochemical data

propose a model in which functional domains, such as the GTPase

domain and the C-terminally located coiled coil structure, act in an

orchestrated manner to coordinate the tethering and mitochondrial

outer membrane fusion. In addition, recent reports shed new light on

the physiological importance of Mitofusin function suggesting a role

in mitochondrial metabolism, apoptosis as well as cellular

signalling. Mutations identified in the human Mfn2 gene from patients

with the peripheral neuropathy Charcot-Marie-Tooth Type 2A invoke a

direct correlation between mitochondrial morphology and function.