FMRP-related retinal phenotypes: Evidence of glutamate-glutamine metabolic cycle impairment

Autor: Maryvonne Ardourel, Arnaud Pâris, Chloé Felgerolle, Fabien Lesne, Isabelle Ranchon-Cole, Sylvain Briault, Olivier Perche
Rok vydání: 2022
Předmět:
Zdroj: Experimental Eye Research. 224:109238
ISSN: 0014-4835
Popis: FMRP, the fragile X mental retardation protein coded by the FMR1 gene, is an RNA-binding protein that assists transport, stabilization and translational regulation of specific synaptic mRNAs. Its expression has been found in multiple cell types of central nervous system (CNS) including glial cells where its involvement in glutamate neurotransmitter homeostasis have been shown. Indeed, glutamate homeostasis deficit has been observed in absence of FMRP in-vivo in cortex and hippocampus structures as well as in vitro on astroglial cell culture. Interestingly, the retina which is an extension of the CNS is presenting electrophysiological alterations in absence of FMRP in both human and murine models suggesting neurotransmitter impairments. Therefore, we investigate the consequences of Fmrp absence on Glutamate-Glutamine cycle in whole retinas and primary retinal Müller cells culture which are the main glial cells of the retina. Using the Fmr1
Databáze: OpenAIRE