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A mouse model brings new perspectives on Lafora disease
Date:12/12/2011

', namely malin and laforin. We now know that when one of these two genes is not active mice develop glycogen aggregates in these cells, which progressively deteriorate and finally die". Since the loss of function of malin induces the accumulation of LB and cell death in neurons in human LD patients and mice, "this model helps us to confirm our hypotheses on the origin of this LD".

Also, "the study provides evidence of the existence of LB in a special type of brain cells, known as inhibitory interneurons". In the complex architecture of the brain, these inhibitory interneurons normally generate 'stop signals' for neural excitation. In this regard, the researchers propose that the higher excitability of neurons and the propensity to epileptic seizures observed in these engineered mice are related to the loss of inhibitory interneurons. Interestingly, decreases in the populations of this cell type occur in some cases of epilepsy, schizophrenia, and some neurodegenerative disorders, such as Alzheimer's.

Scarcely a hundred years have passed since the Spanish neurologist Gonzalo R. Lafora described Lafora bodies. Yet unravelling the origins of LD is on the horizon. A better understanding of the mechanisms that block glycogen accumulation in brain cells will provide new insights towards effective treatments for LD and other neurodegenerative diseases.


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Contact: Nuria Noriega
34-934-037-255
Institute for Research in Biomedicine (IRB Barcelona)
Source:Eurekalert

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