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She will have less severe symptoms. if the reverse happens the child will have more severe symptoms. although rare, it is also possible for males to have mecp2 mutations. the symptoms can range from bipolar disease and mental retardation to very sick infants who die in their first year. dr. zoghbi has designed, through genetic engineering, a mouse model that mimics the human disorder. the model has a mutation that causes the mecp2 protein to be abnormally shortened. the mice have a normal first month of life and then develop tremors, spasticity, hypoactivity, seizures, ataxia, paw stereotypies, social behavior dysfunction, and kyphosis. the zoghbi lab has analyzed in detail the social behaviors of the mutant mice and found that the animals had abnormalities in nest building as well as decreased nest usage. the mutant mice also took less initiative and were less decisive in approaching unfamiliar males. interaction with novel inanimate objects, however, was normal. these mice will be very helpful in identifying the molecular basis of social impairment in rtt. dr. zoghbi touched on the work of rudolf jaenisch at whitehead institute. earlier this year, with rsrf funding, dr. jaenisch genetically engineered mice so that healthy mecp2 protein would be introduced when the brain cells became mature approximately at birth ; . the genetically engineered mice were "rescued" from ever developing rett-like symptoms. from this, dr. jaenisch concludes that children born with rett syndrome probably have healthy neurons and if treatments were available it is likely these newborns continued from page 16 ; lastly, dr. zoghbi discussed her collaboration with nathaniel heintz of rockefeller university. with rsrf's funding, this work will hopefully yield neuron-specific gene changes in mutant mice as compared to controls. once these are identified, researchers can address questions regarding the mechanism by which mecp2 causes specific neuronal abnormalities and identify pathways that may be targeted pharmacologically. a lively q&a session followed dr. zoghbi's presentation. monica coenraads, director of research for rsrf, then spoke briefly about rsrf's research program and future goals. rsrf would like to thank dr. zoghbi for taking time out of her busy schedule to make this presentation. we would like to also thank all of the wonderful families who attended. would never manifest the symptoms of rett syndrome. dr. zoghbi discussed ongoing experiments in her lab in which mice were generated that expressed higher than normal levels of mecp2. interestingly these mice, at around 1220 weeks of age, displayed enhanced learning. by 1 year of age, however, they all succumbed to neurological problems including seizures, ataxia, hypoactivity, kyphosis and finally death. the lab then mated the mice which overexpressed mecp2 with mutant mice lacking mecp2 generated in dr. bird's lab ; . to date, the offspring have not manifested any detectable motor or behavioral abnormalities. through this work it is possible to conclude that levels of mecp2 are tightly regulated and critical to neurological function. too much mecp2 is just as detrimental as too little. Winter, starved to madness, and had begun eating two cows in their shed, when little ljubov chanced upon them and was discovered half an hour later, surrounded by two hundred of these wolves, which were pushing and kicking one another to lick her hands. on another occasion, extraordinary miracle, one glance from her eyes had stopped the tongue of a drunken pope who was swearing at a peasant in the foulest language. she was, of course, a favourite with all in the village: the simpler and nearer nature their souls, the more they gave the child her proper place. but it must not be inferred that little ljubov was either worshipped or freed from such menial works as children of her age are called upon to perform. nor did her playmates realize her superiority. the alleged miracles and the reported cases of healing were heard of some ten years after her death, when eye-witnesses had all departed from this world. yet, of course, they were possible, quite possible, quite. iii "all of you, suckling babes, have read the russian tale of the man who bought souls--or heard of it. men of a similar turn of mind exist in russia, and i want you to concentrate your mind upon such a man, albeit his bargains cost him even less, and were of a more physical reality. from town to village he went, in search of treasures in the shape of eyes. the tools of his trade were a few walnut shells, enamelled within, and a certain magical liquid preparation, which he used to preserve the qualities, freshness and beauty of his acquisitions. 297.