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  • A gentle death for neurons?

    images92A recent study published in Nature Neuroscience reveals that excess glycogen induces neuronal death and causes a fatal kind of epilepsy that affects adolescents. This previously unknown mechanism could be key to understanding other neurodegenerative diseases, as is clear from research coordinated by Joan Guinovart Institute of Research in Biomedicine (IRB Barcelona) and Santiago Rodríguez de Córdoba Biological Research Center of CSIC .


    The discovery has been made studying Lafora disease, a disorder that causes irreversible neurodegeneration in adolescents and for which no treatment exists. Lafora disease is manifested in the form of initial and subsequent seizures myoclonus leading to a terminal vegetative state ten years after its appearance. It is transmitted as inherited through parents carry mutations in one of two genes that have been associated with the disease (laforin and malin.) The condition is characterized by the accumulation in neurons of some abnormal glycogen deposits called “Lafora bodies.”


    This paper has described the function of laforin and malin, and explains the origin of Lafora bodies and identifies how the neurodegenerative process arises from the disease. Normally, the synthesis of glycogen is absent in neurons, but not for lack of the enzymatic machinery responsible for it, but because the synthesizing machinery is inactivated by the action of a complex formed by the suppressor gene products laforin and malin, which stimulates the degradation of proteins responsible for the accumulation of glucose as glycogen. When one of the two genes loses its function by mutation, there is no destruction of these proteins, glycogen accumulates and deteriorates the neurons and induce cell suicide mechanism for apoptosis.

    Published on December 7, 2012 · Filed under: Bioscience; Tagged as: , , ,
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