Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice

Neurobiology of Disease
Mervi KuronenOuti Kopra

Abstract

The neuronal ceroid lipofuscinoses constitute the most common group of childhood neurodegenerative disorders. These devastating disorders still remain without effective treatment. The use of animal models has provided significant information about NCL pathogenesis, highlighting early glial activation and neuron loss in specific brain regions of affected animals. Here, we have characterized the timing and regional-specificity of the pathological events of CLN8 disease utilizing the Cln8 deficient mouse model, Cln8(mnd). We have studied the progression of neuron loss, astrocytosis and microglial activation from early to moderately symptomatic (1, 3 and 5 months) and late symptomatic (8 months) mice. In Cln8 deficiency, the somatosensory pathway comprising the thalamic ventral posterior nucleus (VPM/VPL) and the primary somatosensory cortex (S1BF) was found to be the most affected relay system. Scattered microglia that appeared partially activated were already present at 3 months of age, followed by astrocytosis and the loss of thalamic relay neurons at 5 months of age, with all these phenotypes and glial activation becoming more pronounced with disease progression. Reactive changes followed a similar pattern in the corresponding ...Continue Reading

References

Jan 1, 1979·European Neurology·W Tackmann, D Kuhlendahl
May 1, 1991·Neuropediatrics·P SantavuoriK Sainio
Sep 1, 1987·Journal of Microscopy·H J Gundersen, E B Jensen
Nov 1, 1986·Journal of Neurogenetics·A Messer, L Flaherty
Sep 29, 1966·The New England Journal of Medicine·J Lister
Nov 1, 1982·Journal of Neurology, Neurosurgery, and Psychiatry·A VercruyssenL Swerts
Jan 1, 1997·Acta Neurologica Scandinavica·A H LangJ Siivola
May 5, 1998·Neurology·T MimaH Shibasaki
Oct 31, 2002·Journal of Neuroscience Research·Tiziana MenniniCaterina Bendotti
Dec 21, 2002·The European Journal of Neuroscience·M BertaminiD Curti
May 4, 2006·Brain : a Journal of Neurology·Eija SiintolaJaana Tyynelä
Oct 7, 2006·Biochimica Et Biophysica Acta·Jonathan D CooperHannah M Mitchison
Jun 15, 2007·Pediatric Neurology·Nathanel ZelnikMarsha Zeigler
Jun 15, 2007·American Journal of Human Genetics·Eija SiintolaAnna-Elina Lehesjoki
Oct 24, 2007·Trends in Neurosciences·Knut BiberHendrikus W G M Boddeke
Dec 20, 2007·Journal of Neuropathology and Experimental Neurology·Sanna PartanenJaana Tyynelä

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Citations

Oct 16, 2012·Disease Models & Mechanisms·Tea BlomAnu Jalanko
Jul 16, 2014·Acta Neuropathologica Communications·Yoshinori TanakaMasugi Nishihara
Sep 10, 2014·Acta Neuropathologica Communications·Yulia GrishchukSusan A Slaugenhaupt
Apr 11, 2014·NeuroImage·Anna E MechlingLaura-Adela Harsan
Jun 1, 2015·Biochimica Et Biophysica Acta·Jonathan D CooperHemanth R Nelvagal
Sep 19, 2014·Biochemical Society Transactions·Charles Shyng, Mark S Sands
Jun 4, 2015·Neuroscience·K V Rama Rao, T Kielian
Jan 23, 2013·Biochimica Et Biophysica Acta·Michael BondClaire Russell
Jan 16, 2014·Neurobiology of Disease·Markus DammeStephan Storch
Oct 19, 2017·Acta Neuropathologica Communications·Lotta ParviainenJonathan D Cooper
Nov 9, 2018·Frontiers in Neuroanatomy·Daniel KellerHenry Markram
Oct 5, 2020·Molecular Therapy : the Journal of the American Society of Gene Therapy·Tyler B JohnsonJill M Weimer
Nov 30, 2019·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Robert J HuberClaire Russell
Nov 5, 2019·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Hemanth R NelvagalJonathan D Cooper

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