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Article

Suppression of leptin signaling reduces polyglucosan inclusions and seizure susceptibility in a mouse model for Lafora disease.

Human molecular genetics - 15 Dec 2017

Rai Anupama, Mishra Rohit, Ganesh Subramaniam

Abstract excerpt

Lafora disease (LD) represents a fatal form of neurodegenerative disorder characterized by the presence of abnormally large number of polyglucosan bodies-called the Lafora bodies-in neurons and other tissues of the affected patients. The disease is caused by defects in the EPM2A gene coding for a protein phosphatase (laforin) or the NHLRC1 gene coding for an ubiquitin ligase (malin). Studies have shown that...

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PMID: 28973665
DOI: 10.1093/hmg/ddx357

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