Mirra, SerenaGarcía-Arroyo, RocíoDomènech, Elena B.Gavaldà i Navarro, AleixHerrera Úbeda, CarlosOliva, ClaraGarcia Fernández, JordiArtuch Iriberri, RafaelVillarroya i Gombau, FrancescMarfany i Nadal, Gemma2021-06-282022-05-252021-05-250969-9961https://hdl.handle.net/2445/178699The retina is a highly active metabolic organ that displays a particular vulnerability to genetic and environmental factors causing stress and homeostatic imbalance. Mitochondria constitute a bioenergetic hub that coordinates stress response and cellular homeostasis, therefore structural and functional regulation of the mitochondrial dynamic network is essential for the mammalian retina. CERKL (ceramide kinase like) is a retinal degeneration gene whose mutations cause Retinitis Pigmentosa in humans, a visual disorder characterized by photoreceptors neurodegeneration and progressive vision loss. CERKL produces multiple isoforms with a dynamic subcellular localization. Here we show that a pool of CERKL isoforms localizes at mitochondria in mouse retinal ganglion cells. The depletion of CERKL levels in CerklKD/KO (knockdown/knockout) mouse retinas cause increase of autophagy, mitochondrial fragmentation, alteration of mitochondrial distribution, and dysfunction of mitochondrial-dependent bioenergetics and metabolism. Our results support CERKL as a regulator of autophagy and mitochondrial biology in the mammalian retina.16 p.application/pdfengcc-by-nc-nd (c) Elsevier, 2021https://creativecommons.org/licenses/by-nc-nd/4.0/Malalties de la retinaMitocondrisMamífersRetinal diseasesMitochondriaMammalsinfo:eu-repo/semantics/article7129762021-06-28info:eu-repo/semantics/openAccess