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: While 4-month-old KO brains showed mainly astrocyte-associated LB accumulation (Figs 5 and 6A), 11-month-old counterparts showed LBs in astrocytes (Figs 5 and 6A) and in the cell bodies of neurons (Fig 5). Neuronal LBs were very conspicuous in the neuronal somata of hippocampal PV+ interneurons and were occasionally found in their dendritic processes (Fig 6B).
Affiliation: Institute for Research in Biomedicine (IRB Barcelona) Barcelona, Spain.