Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease.
Bottom Line: They also had LBs in the soma and some processes of PV(+) interneurons.This phenomenon was accompanied by the progressive loss of these neuronal cells and, importantly, neurophysiological alterations potentially related to impairment of hippocampal function.Our results emphasize the relevance of the laforin-malin complex in the control of glycogen metabolism and highlight altered glycogen accumulation as a key contributor to neurodegeneration in LD.
Affiliation: Institute for Research in Biomedicine (IRB Barcelona) Barcelona, Spain.Show MeSH
Related in: MedlinePlus
Mentions: The histological study of mouse brains with antibodies against MGS showed that, in addition to astrocytes, PV+ interneurons of the hippocampus also express MGS (Fig 4A). These cells can be found in the DG, CA1-2 and CA3 (not shown).
Affiliation: Institute for Research in Biomedicine (IRB Barcelona) Barcelona, Spain.