Limits...
Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease.

Valles-Ortega J, Duran J, Garcia-Rocha M, Bosch C, Saez I, Pujadas L, Serafin A, Cañas X, Soriano E, Delgado-García JM, Gruart A, Guinovart JJ - EMBO Mol Med (2011)

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.

View Article: PubMed Central - PubMed

Affiliation: Institute for Research in Biomedicine (IRB Barcelona) Barcelona, Spain.

Show MeSH

Related in: MedlinePlus

Hippocampal interneurons and astrocytes express MGS and malinHippocampal astrocytes and interneurons from dentate gyrus (DG) and CA1 regions of WT (A) and malin heterozygous (B) hippocampi of same-aged mice are shown.Immunostaining with an antibody against MGS (brown).Representative orthogonal confocal sections showing immunostaining with antibodies against glial fibrillary acidic protein (GFAP) (red), parvalbumin (PV) (magenta) and βgal (cyan). Scale bar = 20 µm.
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC3377110&req=5

fig04: Hippocampal interneurons and astrocytes express MGS and malinHippocampal astrocytes and interneurons from dentate gyrus (DG) and CA1 regions of WT (A) and malin heterozygous (B) hippocampi of same-aged mice are shown.Immunostaining with an antibody against MGS (brown).Representative orthogonal confocal sections showing immunostaining with antibodies against glial fibrillary acidic protein (GFAP) (red), parvalbumin (PV) (magenta) and βgal (cyan). Scale bar = 20 µm.

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).


Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease.

Valles-Ortega J, Duran J, Garcia-Rocha M, Bosch C, Saez I, Pujadas L, Serafin A, Cañas X, Soriano E, Delgado-García JM, Gruart A, Guinovart JJ - EMBO Mol Med (2011)

Hippocampal interneurons and astrocytes express MGS and malinHippocampal astrocytes and interneurons from dentate gyrus (DG) and CA1 regions of WT (A) and malin heterozygous (B) hippocampi of same-aged mice are shown.Immunostaining with an antibody against MGS (brown).Representative orthogonal confocal sections showing immunostaining with antibodies against glial fibrillary acidic protein (GFAP) (red), parvalbumin (PV) (magenta) and βgal (cyan). Scale bar = 20 µm.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3377110&req=5

fig04: Hippocampal interneurons and astrocytes express MGS and malinHippocampal astrocytes and interneurons from dentate gyrus (DG) and CA1 regions of WT (A) and malin heterozygous (B) hippocampi of same-aged mice are shown.Immunostaining with an antibody against MGS (brown).Representative orthogonal confocal sections showing immunostaining with antibodies against glial fibrillary acidic protein (GFAP) (red), parvalbumin (PV) (magenta) and βgal (cyan). Scale bar = 20 µm.
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).

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.

View Article: PubMed Central - PubMed

Affiliation: Institute for Research in Biomedicine (IRB Barcelona) Barcelona, Spain.

Show MeSH
Related in: MedlinePlus