Contos J J, Fukushima N, Weiner J A, Kaushal D, Chun J
Department of Pharmacology, Neuroscience Graduate Program, Biomedical Sciences Program, School of Medicine, University of California, San Diego, La Jolla, CA 92093-0636, USA.
Proc Natl Acad Sci U S A. 2000 Nov 21;97(24):13384-9. doi: 10.1073/pnas.97.24.13384.
Although extracellular application of lysophosphatidic acid (LPA) has been extensively documented to produce a variety of cellular responses through a family of specific G protein-coupled receptors, the in vivo organismal role of LPA signaling remains largely unknown. The first identified LPA receptor gene, lp(A1)/vzg-1/edg-2, was previously shown to have remarkably enriched embryonic expression in the cerebral cortex and dorsal olfactory bulb and postnatal expression in myelinating glia including Schwann cells. Here, we show that targeted deletion of lp(A1) results in approximately 50% neonatal lethality, impaired suckling in neonatal pups, and loss of LPA responsivity in embryonic cerebral cortical neuroblasts with survivors showing reduced size, craniofacial dysmorphism, and increased apoptosis in sciatic nerve Schwann cells. The suckling defect was responsible for the death among lp(A1)((-/-)) neonates and the stunted growth of survivors. Impaired suckling behavior was attributable to defective olfaction, which is likely related to developmental abnormalities in olfactory bulb and/or cerebral cortex. Our results provide evidence that endogenous lysophospholipid signaling requires an lp receptor gene and indicate that LPA signaling through the LP(A1) receptor is required for normal development of an inborn, neonatal behavior.
尽管已有大量文献记载,细胞外施用溶血磷脂酸(LPA)可通过一系列特定的G蛋白偶联受体产生多种细胞反应,但LPA信号传导在体内生物体中的作用仍 largely未知。首个被鉴定出的LPA受体基因lp(A1)/vzg-1/edg-2,此前已显示在大脑皮层和背侧嗅球中胚胎表达显著富集,在包括雪旺细胞在内的髓鞘形成胶质细胞中出生后表达。在此,我们表明,靶向缺失lp(A1)会导致约50%的新生仔鼠死亡、新生幼崽吮乳受损,以及胚胎大脑皮层神经母细胞中LPA反应性丧失,存活者表现出体型减小、颅面畸形,坐骨神经雪旺细胞凋亡增加。吮乳缺陷是lp(A1)((-/-))新生仔鼠死亡及存活者生长发育迟缓的原因。吮乳行为受损归因于嗅觉缺陷,这可能与嗅球和/或大脑皮层的发育异常有关。我们的结果提供了证据,表明内源性溶血磷脂信号传导需要一个lp受体基因,并表明通过LP(A1)受体的LPA信号传导是先天性新生行为正常发育所必需的。