Mitochondrial Proteins Implicated in Neurodegenerative Disorders

Nervous-system researchers have found that the slow Wallerian degeneration gene modifies the expression of at least 16 proteins including eight that are regulators of mitochondrial stability and degeneration.

When a nerve axon is cut or crushed, the nerve fibers in the distal part of the axon, separated from the cell body, undergo a form of spontaneous degeneration, known as Wallerian degeneration. Furthermore, neurons are primary pathologic targets in many neurodegenerative conditions, ranging from Alzheimer disease to motor neuron disease. Axons and synapses are protected from degeneration by the slow Wallerian degeneration (Wlds) gene.

Investigators at the University of Edinburgh (UK) used differential proteomics analysis to identify proteins whose expression levels were significantly altered in isolated synaptic preparations from the striatum of Wlds mice. Results published in the August 2007 issue of the journal Molecular & Cellular Proteomics revealed that eight of the 16 proteins that modified expression levels in Wlds synapses were known regulators of mitochondrial stability and degeneration. Of the non-mitochondrial proteins identified, several had been implicated in neurodegenerative diseases where synapses and axons were primary pathologic targets.

The investigators concluded that, "Altered mitochondrial responses to degenerative stimuli are likely to play an important role in the neuroprotective Wlds phenotype and that targeting proteins identified in the current study may lead to novel therapies for the treatment of neurodegenerative diseases in humans.”


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