Systems Neuroscience Group
Neuronal Pathophysiology
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Lionel Muller Igaz
Independent Researcher, CONICET
Teaching Assistant, UBA
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The Laboratory of Neuronal Pathophysiology at IFIBIO is engaged in research lines that integrate multidisciplinary approaches, including behavioral, pharmacological, biochemical, and molecular studies, to understand the pathogenesis of neurodegenerative diseases. This understanding is crucial for developing new and more effective therapies. Specifically, our focus is on TDP-43 proteinopathies, including Amyotrophic Lateral Sclerosis (ALS), Frontotemporal Dementia (FTD), and other diseases related to TDP-43 protein alterations. We utilize cellular and animal models, including transgenic mice, to investigate these conditions.
Members
Postdoctoral fellow
Conicet
Contact
Role of TDP-43 in modulating the unfolded protein response (UPR) and the therapeutic potential of UPR modulation in pathologies such as ALS and FTD (Frontotemporal Dementia).
Doctoral fellow
Conicet
Contact
Degenerative and inflammatory processes mediated by TDP-43 in vivo: The role of glial activation.
Doctoral fellow
Foncyt
Contacto
Role of changes in neuronal activation/plasticity in behavioral deficits mediated by TDP-43 alterations.
Selected Publications
- Protein synthesis modulation as a therapeutic approach for amyotrophic lateral sclerosis and frontotemporal dementia. Charif SE, Vassallu MF, Salvañal L, Igaz LM. Neural Regen Res. 2022 Jul;17(7):1423-1430. doi: 10.4103/1673-5374.330593.
- Cytoplasmic expression of the ALS/FTD-related protein TDP-43 decreases global translation both in vitro and in vivo. Charif SE*, Luchelli L*, Vila A, Blaustein M, Igaz LM. Frontiers in Cellular Neuroscience 2020 Dec 8;14:594561. doi: 10.3389/fncel.2020.594561.
- Suppression of conditional TDP-43 transgene expression differentially affects early cognitive and social phenotypes in TDP-43 mice. Silva PR, Nieva GV, Igaz LM. Frontiers in Genetics. 2019, Apr 24;10:369. doi: 10.3389/fgene.2019.00369.
- Early cognitive/social deficits and late motor phenotype in conditional wild-type TDP-43 transgenic mice. Alfieri JA, Silva PR, Igaz LM. Frontiers in Aging Neuroscience 2016 Dec 6. 8:310. doi: 10.3389/fnagi.2016.00310.
- Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies. Alfieri JA, Pino NS, Igaz LM. The Journal of Neuroscience. 2014 Nov 12;34(46):15244-59. doi: 10.1523/JNEUROSCI.1918-14.2014.
- Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. Igaz LM*, Kwong LK*, Lee EB*, Chen-Plotkin A, Swanson E, Unger T, Malunda J, Xu Y, Winton MJ, Trojanowski JQ, Lee VM. The Journal of Clinical Investigation. 2011 Feb 1;121(2):726-38. Epub 2011 Jan 4.
- Expression of TDP-43 C-terminal fragments in vitro recapitulates pathological features of TDP-43 proteinopathies. Igaz LM, Kwong LK, Chen-Plotkin A, Winton MJ, Unger TL, Xu Y, Neumann M, Trojanowski JQ, Lee VM. Journal of Biological Chemistry. 2009 Mar 27;284(13):8516-24. Epub 2009 Jan 21.