CMT 2A: Neurodegenerative Disease Cell Biology

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Neurodegenerative Disease Cell Biology

http://www.newsrx.com/articles/707746.html

(NewsRx.com) -- Fresh data on neurodegenerative disease are

presented in the report " Mitochondrial fusion protects against

neurodegeneration in the cerebellum. " According to recent research

from the United States, " Mutations in the mitochondrial fusion gene

Mfn2 cause the human neurodegenerative disease Charcot-Marie-Tooth

type 2A. However, the cellular basis underlying this relationship is

poorly understood. "

" By removing Mfn2 from the cerebellum, we established a model for

neurodegeneration caused by loss of mitochondrial fusion. During

development and after maturity, Purkinje cells require Mfn2 but not

Mfn1 for dendritic outgrowth, spine formation, and cell survival. In

vivo, cell culture, and electron microscopy studies indicate that

mutant Purkinje cells have aberrant mitochondrial distribution,

ultrastructure, and electron transport chain activity. In

fibroblasts lacking mitochondrial fusion, the majority of

mitochondria lack mitochondrial DNA nucleoids. This deficiency

provides a molecular mechanism for the dependence of respiratory

activity on mitochondrial fusion, " wrote H. Chen and colleagues,

California Institute of Technology, Division of Biology.

The researchers concluded: " Our results show that exchange of

mitochondrial contents is important for mitochondrial function as

well as organelle distribution in neurons and have important

implications for understanding the mechanisms of neurodegeneration

due to perturbations in mitochondrial fusion. "

Chen and colleagues published their study in Cell (Mitochondrial

fusion protects against neurodegeneration in the cerebellum. Cell,

2007;130(3):548-62).