Mutations in the MFN2 gene are the most common cause of the neurodegenerative disease Charcot-Marie-Tooth type 2A (CMT2A), but it has been unclear how these defects lead to the selective degeneration of the long peripheral axons that is characteristic of the disorder. Now, a new study shows how defects in the protein encoded by MFN2, mitofusin 2 (Mfn2), can disrupt the movement of mitochondria along nerve cells (Misko A et al. J Neuroscience. 2010;30:4232-4240).
Friedrich MJ. Mitochondrial Mutations. JAMA. 2010;303(17):1686. doi:10.1001/jama.2010.528