Acharya Jairaj K, Dasgupta Ujjaini, Rawat Satinder S, Yuan Changqing, Sanxaridis Parthena D, Yonamine Ikuko, Karim Pusha, Nagashima Kunio, Brodsky Michael H, Tsunoda Susan, Acharya Usha
Laboratory of Cell and Developmental Signaling, National Cancer Institute, Frederick, MD 21702, USA.
Neuron. 2008 Jan 10;57(1):69-79. doi: 10.1016/j.neuron.2007.10.041.
Neutral ceramidase, a key enzyme of sphingolipid metabolism, hydrolyzes ceramide to sphingosine. These sphingolipids are critical structural components of cell membranes and act as second messengers in diverse signal transduction cascades. Here, we have isolated and characterized functional null mutants of Drosophila ceramidase. We show that secreted ceramidase functions in a cell-nonautonomous manner to maintain photoreceptor homeostasis. In the absence of ceramidase, photoreceptors degenerate in a light-dependent manner, are defective in normal endocytic turnover of rhodopsin, and do not respond to light stimulus. Consistent with a cell-nonautonomous function, overexpression of ceramidase in tissues distant from photoreceptors suppresses photoreceptor degeneration in an arrestin mutant and facilitates membrane turnover in a rhodopsin null mutant. Furthermore, our results show that secreted ceramidase is internalized and localizes to endosomes. Our findings establish a role for a secreted sphingolipid enzyme in the regulation of photoreceptor structure and function.
中性神经酰胺酶是鞘脂代谢的关键酶,可将神经酰胺水解为鞘氨醇。这些鞘脂是细胞膜的关键结构成分,并在多种信号转导级联反应中作为第二信使发挥作用。在此,我们分离并鉴定了果蝇神经酰胺酶的功能缺失突变体。我们发现分泌型神经酰胺酶以非细胞自主方式发挥作用,以维持光感受器的稳态。在缺乏神经酰胺酶的情况下,光感受器以光依赖的方式退化,视紫红质的正常内吞周转存在缺陷,并且对光刺激无反应。与非细胞自主功能一致,在远离光感受器的组织中过表达神经酰胺酶可抑制 Arrestin 突变体中的光感受器退化,并促进视紫红质缺失突变体中的膜周转。此外,我们的结果表明,分泌型神经酰胺酶被内化并定位于内体。我们的研究结果确立了分泌型鞘脂酶在调节光感受器结构和功能中的作用。