CERKL, a retinal dystrophy gene, regulates mitochondrial function and dynamics in the mammalian retina

CERKL, a retinal dystrophy gene, regulates mitochondrial function and dynamics in the mammalian retina

Blog Article

The 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 Wax Melt Burner 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 Gas Door Cover as a regulator of autophagy and mitochondrial biology in the mammalian retina.