Teillet M A, Naquet R, Le Gal La Salle G, Merat P, Schuler B, Le Douarin N M
Institut d'Embryologie Cellulaire et Moléculaire du Centre National de la Recherche Scientifique et du Collège de France, Nogent-sur-Marne.
Proc Natl Acad Sci U S A. 1991 Aug 15;88(16):6966-70. doi: 10.1073/pnas.88.16.6966.
In the Fayoumi chicken, a spontaneous recessive autosomal mutation (F.Epi) is responsible for high susceptibility to seizures that are especially inducible by intermittent light stimulation. Substitution of defined areas of the encephalic neuroepithelium in normal chicken embryos at 2 days of incubation by their counterparts from homozygous F.Epi embryos generates the epileptic phenotype in the chimeras. It was found that grafting primordia of both prosencephalon and mesencephalon of homozygous F.Epi birds is necessary and sufficient for transfer of the full disease. When grafted alone, the homozygous F.Epi prosencephalon, although showing the typical epileptic interictal electroencephalogram, does not allow the complete epileptic seizures to occur in the hosts. Grafts of mesencephalon and/or rhombencephalon modify neither the behavior nor the electroencephalographic pattern of the recipient chickens. Cooperation of forebrain and midbrain activities is therefore required to yield epileptic seizures in this model.
在法尤米鸡中，一种自发的隐性常染色体突变（F.Epi）导致其对癫痫发作高度易感，这种发作尤其可由间歇性光刺激诱发。在孵化2天的正常鸡胚中，用来自纯合F.Epi胚胎的相应部位替换脑神经性上皮的特定区域，可在嵌合体中产生癫痫表型。研究发现，移植纯合F.Epi鸟类的前脑和中脑原基对于完全传递疾病是必要且充分的。单独移植时，纯合F.Epi前脑虽然显示出典型的癫痫发作间期脑电图，但不会使宿主发生完全的癫痫发作。中脑和/或后脑的移植既不改变受体鸡的行为，也不改变其脑电图模式。因此，在该模型中，前脑和中脑活动的协同作用是产生癫痫发作所必需的。
