Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA; Manton Center for Orphan Disease, Boston Children's Hospital, Boston, MA, USA.
Center for Translational Vision Research, Gavin Herbert Eye Institute, Irvine, CA, USA.
J Lipid Res. 2021;62:100035. doi: 10.1194/jlr.TR120000618. Epub 2021 Feb 6.
Photoreceptors have high energy demands and a high density of mitochondria that produce ATP through oxidative phosphorylation (OXPHOS) of fuel substrates. Although glucose is the major fuel for CNS brain neurons, in photoreceptors (also CNS), most glucose is not metabolized through OXPHOS but is instead metabolized into lactate by aerobic glycolysis. The major fuel sources for photoreceptor mitochondria remained unclear for almost six decades. Similar to other tissues (like heart and skeletal muscle) with high metabolic rates, photoreceptors were recently found to metabolize fatty acids (palmitate) through OXPHOS. Disruption of lipid entry into photoreceptors leads to extracellular lipid accumulation, suppressed glucose transporter expression, and a duel lipid/glucose fuel shortage. Modulation of lipid metabolism helps restore photoreceptor function. However, further elucidation of the types of lipids used as retinal energy sources, the metabolic interaction with other fuel pathways, as well as the cross-talk among retinal cells to provide energy to photoreceptors is not fully understood. In this review, we will focus on the current understanding of photoreceptor energy demand and sources, and potential future investigations of photoreceptor metabolism.
感光器的能量需求很高,线粒体密度也很高,这些线粒体通过氧化磷酸化(OXPHOS)作用来利用燃料底物产生 ATP。尽管葡萄糖是中枢神经系统(CNS)脑神经元的主要燃料,但在感光器(也是 CNS)中,大部分葡萄糖不是通过 OXPHOS 代谢,而是通过有氧糖酵解转化为乳酸。感光器线粒体的主要燃料来源在近六十年间仍不明确。与其他代谢率较高的组织(如心脏和骨骼肌)类似,最近发现感光器通过 OXPHOS 代谢脂肪酸(棕榈酸)。脂质进入感光器的中断会导致细胞外脂质积累、葡萄糖转运蛋白表达抑制以及双重的脂质/葡萄糖燃料短缺。脂质代谢的调节有助于恢复感光器功能。然而,对于用作视网膜能量来源的脂质类型、与其他燃料途径的代谢相互作用以及为感光器提供能量的视网膜细胞之间的串扰等方面的进一步阐明仍不完全清楚。在这篇综述中,我们将重点介绍当前对感光器能量需求和来源的理解,以及对感光器代谢的潜在未来研究。