"This discovery places this type of CMT in the ever-growing list of neurodegenerative diseases caused by transport problems and strengthens the possibility of using general enhancers of this process as therapy for different types of diseases," said Vincent Timmerman, PhD, of the University of Antwerp in Belgium, who was unaffiliated with the study.
The authors also suggest that a related protein called mitofusin 1 might someday serve to compensate for a mutated and malfunctioning mitofusin 2. Although mitofusins 1 and 2 are different proteins, they play similar roles in a cell. Baloh and his team suggest that mitofusin 1 may be able to perform the function of mitofusin 2 and regulate the transport of mitochondria. Finding a way to increase the levels of mitofusin 1 might have therapeutic effects for patients who have mutated mitofusin 2.
This pair of time-lapse movies show mitochondria moving along nerve cell fibers. In the first clip, mitochondria with normal mitofusin proteins move quickly along the fiber. In this second clip, mitochondria with a mutant form of mitofusin 2 are smaller and move slowly. This abnormal movement is thought to be the basis for the neurodegenerative disease Charcot-Marie-Tooth type 2A.
(Photo Credit: Courtesy, with permission: Misko et al. The Journal of Neuroscience 2010.)
This pair of time-lapse movies show mitochondria moving along nerve cell fibers. In this clip, mitochondria with normal mitofusin proteins move quickly along the fiber. In the second clip, mitochondria with a mutant form of mitofusin 2 are smaller and move slowly. This abnormal movement is thought to be the basis for the neurodegenerative disease Charcot-Marie-Tooth type 2A.
(Photo Credit: Courtesy, with permission: Misko et al. The Journal of Neuroscience 2010.)
Cells with a mutant form of the protein mitofusin 2 contain mitochondria that are smaller and move more slowly than those with normal proteins. This graph shows how much more normal mitochondria move over time (top graph) compared with how little mitochondria with mutated proteins travel (bottom graph).
(Photo Credit: Courtesy, with permission: Misko et al. The Journal of Neuroscience 2010.)
Source: Society for Neuroscience