Pérez-Fígares J M, Jiménez A J, Pérez-Martín M, Fernández-Llebrez P, Cifuentes M, Riera P, Rodríguez S, Rodríguez E M
Departamento de Biología Celular y Genética, Facultad de Ciencias, Universidad de Málaga, Spain.
J Neuropathol Exp Neurol. 1998 Feb;57(2):188-202. doi: 10.1097/00005072-199802000-00010.
The subcommissural organ is an ependymal gland located at the entrance of the cerebral aqueduct. It secretes glycoproteins into the cerebrospinal fluid, where they aggregate to form Reissner's fiber. This fiber grows along the aqueduct, fourth ventricle, and central canal. There is evidence that the subcommissural organ is involved in the pathogenesis of congenital hydrocephalus. This organ was investigated in the mutant mouse hyh developing a congenital hydrocephalus. The central nervous system of normal and hydrocephalic hyh mice, 1 to 40 days old, was investigated using antibodies recognizing the subcommissural organ secretory glycoproteins, and by transmission and scanning electron microscopy. At birth, the affected mice displayed open communications between all ventricles, absence of a central canal in the spinal cord, ependymal denudation of the ventricles, stenosis of the rostral end of the aqueduct, and hydrocephalus of the lateral and third ventricles and of the caudal end of the aqueduct. Around the 5th postnatal day, the communication between the caudal aqueduct and fourth ventricle sealed, and hydrocephalus became severe. It is postulated that the hyh mice carry a genetic defect affecting the ependymal cell lineage. The subcommissural organ showed signs of increased secretory activity; it released to the stenosed aqueduct a material that aggregated, but it did not form a Reissner's fiber. A large area of the third ventricular wall differentiated into a secretory ependyma synthesizing a material similar to that secreted by the subcommissural organ. It is concluded that the subcommissural organ changes during hydrocephalus; whether these changes precede hydrocephalus needs to be investigated.
连合下器官是位于中脑导水管入口处的一个室管膜腺。它向脑脊液中分泌糖蛋白，这些糖蛋白聚集形成赖氏纤维。该纤维沿着导水管、第四脑室和中央管生长。有证据表明连合下器官参与先天性脑积水的发病机制。在发生先天性脑积水的突变小鼠hyh中对该器官进行了研究。使用识别连合下器官分泌性糖蛋白的抗体，以及通过透射电子显微镜和扫描电子显微镜，对1至40日龄的正常和脑积水hyh小鼠的中枢神经系统进行了研究。出生时，受影响的小鼠所有脑室之间存在开放连通，脊髓中无中央管，脑室室管膜剥脱，导水管头端狭窄，侧脑室、第三脑室以及导水管尾端出现脑积水。出生后第5天左右，导水管尾端与第四脑室之间的连通封闭，脑积水变得严重。据推测，hyh小鼠携带影响室管膜细胞谱系的遗传缺陷。连合下器官显示出分泌活性增加的迹象；它向狭窄的导水管释放一种聚集的物质，但未形成赖氏纤维。第三脑室壁的大片区域分化为分泌性室管膜，合成一种与连合下器官分泌的物质相似的物质。得出的结论是，连合下器官在脑积水过程中发生变化；这些变化是否先于脑积水尚需研究。
