How to stop the suicide of neurons
Dulbecco Telethon Institute conducted a study in Padua suggests how to avoid the death of nerve cells in Huntington’s disease, severe neurodegenerative disease.
New light on the mechanisms that lead to nerve cell death in patients with Huntington’s disease: a show on the pages of EMBO Molecular Medicine * is Luca Scorrano, Dulbecco Telethon Institute researcher who worked in Padua at the Venetian Institute of Molecular Medicine.
Huntington’s disease is a severe degenerative disease of the nervous system, although hereditary, begins to manifest only in adulthood. Who is affected initially shows problems in coordination and movement control, and impaired concentration, memory and language, to the complete dementia.
It is well known for many years that the underlying disease is a defect in the gene that contains the information for a particular protein, called huntingtin in fact, that is altered when it loses its normal functions and becomes toxic to brain cells. The mechanisms by which this happens, however, are not yet entirely clear: what researchers have certainly realized is that the players involved in the degenerative process are many.
Among these are the mitochondria, structures found in all cells responsible for both energy production, and the auditing of “programmed suicide” of cells, which is put in place of the cells in response to specific signals in the body.
The slide assembly has shown that in Huntington’s disease mitochondria show alterations in form and structure that result in actual death signals to neurons. As explained by Veronica Costa, the first author of the work, “we have identified a particular protein, DRP 1, which determines the change in shape of mitochondria: blocking the activity in cells from patients with Huntington’s disease we were able to restore normal structure mitochondria and to arrest the degenerative process. ”
The discovery gives a further contribution to understanding the mechanisms underlying this complex disease, yet without the possibility of cure, but not all. “ORD 1 could be an interesting pharmacological target to block the neurodegeneration typical of chorea,” adds Scorrano. “Better understanding the role in the cell is then our next challenge for those of us who is responsible for basic research, to be echoed as close to the sick as this may give us something extra.”
The work of Luca Scorrano is also supported by Auchan.
* V. Costa, M. Giacomello, R. Hudec, R. Lopreiato, G. Ermak, D. Lim, W. Malorni, K. Davies, E. Carafoli, L. Scorrano, “Mitochondrial fission and cristae disruption Increase the response of cell models of Huntington’s disease to apoptotic stimuli. EMBO Molecular Medicine, 2010.
