Saturday, December 5, 2015

Drosophila study relevant to NTE-related motor neuron disorder reveals possible contribution of glia to pathology of the disease

Dutta S, Rieche F, Eckl N, Duch C, Kretzschmar D. Glial expression of Swiss-cheese (SWS), the Drosophila orthologue of Neuropathy Target Esterase, is required for neuronal ensheathment and function. Dis Model Mech. 2015 Dec 3. PMID: 26634819.

From the abstract: "Swiss-cheese (SWS) and its vertebrate ortholog Neuropathy Target Esterase (NTE) cause progressive neuronal degeneration in Drosophila and mice and a complex syndrome in humans that includes mental retardation, spastic paraplegia, and blindness. SWS and NTE are widely expressed in neurons but can also be found in glia ... We have used a knockdown approach to specifically address SWS function in glia ... loss of SWS in pseudocartridge glia causes the formation of multi-layered glial whorls in the lamina cortex, the first optic neuropil. This phenotype can be rescued by the expression of SWS and NTE suggesting that the glial function is conserved in the vertebrate protein. ... the loss of SWS in glia impairs neuronal function, thereby playing an important role in the phenotypes described in the sws mutant. It is therefore likely that changes in glia also contribute to the pathology observed in patients that carry mutations in NTE."

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