Journal article
Neuronal pentraxin 1 depletion delays neurodegeneration and extends life in Sandhoff disease mice
Abstract
GM2 gangliosidoses are a group of lysosomal storage disorders which include Sandhoff disease and Tay-Sachs disease. Dysregulation of glutamate receptors has been recently postulated in the pathology of Sandhoff disease. Glutamate receptor association with neuronal pentraxins 1 and 2, and the neuronal pentraxin receptor facilitates receptor potentiation and synaptic shaping. In this study, we have observed an upregulation of a novel form of …
Authors
Hooper AWM; Alamilla JF; Venier RE; Gillespie DC; Igdoura SA
Journal
Human Molecular Genetics, Vol. 26, No. 4, pp. 661–673
Publisher
Oxford University Press (OUP)
Publication Date
February 15, 2017
DOI
10.1093/hmg/ddw422
ISSN
0964-6906