RT journal article
T1 Retinal dysfunction in Huntington's disease mouse models concurs with local gliosis and microglia activation
A1 Cano Cano, Fátima
A1 Martín Loro, Francisco
A1 Gallardo Orihuela, Andrea
A1 González Montelongo, María del Carmen
A1 Ortuño Miguel, Samanta
A1 Hervás-Corpión, Irati
A1 de la Villa, Pedro
A1 Ramón Marco, Lucía
A1 Navarro Calvo, Jorge
A1 Gómez Jaramillo, María Laura
A1 Arroba Espinosa, Ana Isabel
A1 Valor, Luis M.
A2 BiomedicinaBiotecnología y Salud Pública
A2 Medicina
K1 Neuroscience
K1 Diseases of the nervous system
K1 Huntington's disease
AB Huntington's disease (HD) is caused by an aberrant expansion of CAG repeats in the HTT gene that mainly affects basal ganglia. Although striatal dysfunction has been widely studied in HD mouse models, other brain areas can also be relevant to the pathology. In this sense, we have special interest on the retina as this is the most exposed part of the central nervous system that enable health monitoring of patients using noninvasive techniques. To establish the retina as an appropriate tissue for HD studies, we need to correlate the retinal alterations with those in the inner brain, i.e., striatum. We confirmed the malfunction of the transgenic R6/1 retinas, which underwent a rearrangement of their transcriptome as extensive as in the striatum. Although tissue-enriched genes were downregulated in both areas, a neuroinflammation signature was only clearly induced in the R6/1 retina in which the observed glial activation was reminiscent of the situation in HD patient's brains. The retinal neuroinflammation was confirmed in the slow progressive knock-in zQ175 strain. Overall, these results demonstrated the suitability of the mouse retina as a research model for HD and its associated glial activation.
SN 2045-2322
YR 2024
FD 2024-02-20
LK http://hdl.handle.net/10498/31564
UL http://hdl.handle.net/10498/31564
LA eng
DS Repositorio Institucional de la Universidad de Cádiz
RD 09-nov-2026