| Co-Investigator(Kenkyū-buntansha) |
ONODEARA Kazukiyo National Institute of Neuroscience, NCNP,Researcher, 精神センター・神経研究所,2部, 研究員
許斐 博史 国立精神, 精神センター・神経研究所,2部, 研究員 (00186719)
NISHIDA Akira National Institute of Neuroscience, NCNP,Researcher, 精神センター・神経研究所,2部, 研究員
TSURU Akira National Institute of Neuroscience, NCNP,Researcher, 精神センター・神経研究所,2部, 研究員
KONOMI Hiroshi National Institute of Neuroscience, NCNP,Researcher
|
|---|
| Research Abstract |
The brain banking of fetuses to children exceeded 1,000 cases. Using these cases, systemic studies of normal development and developmental disorders were poerformed. In normal brains, the antibodies to gene products of genetic diseases were produced, and their location and function were examined. LIS-1 of Miller-Dieker syndrome and tuberin of tuberous sclerosis were expressed in neurons and glia from early fetal period, and concerned to formation and differentiation of neural cells in the brains. Gart and GARS,and d-seline were also examined biochemically and histochemically. Normal development of N-acetyl-L-aspartic acid in neurons, glutamine recepter sub-units in neurons, glutamate transporters in cerebellar cortex, proteolipid protein in the cerebral white matter was studied, and made a base on the study of developmental disoders and functional disturbances.
In abnormal cases, the pathogenesis of neuronal migration disorders, chromosomal disorders, neurocutaneous syndrome, perinatal cerebrovascular damage, and SIDS was examined. Neonatal pontosubicular neuron necrosis (PSN) showed a transient decrease of superoxide dismutase, which suggests tht free radicals are related to the pathogenesis and repairment of PSN.Periventricular leukomalacia occurred even in extremely low birth weight infants, associated with axon damage, microglial and astrocytic activation. LIS-1 and tuberin were reduced in the brains of lissencephaly and tuberous sclerosis, respectively, and useful for their pathological diagnosis. In Down syndorome, APP and Etz gene protein were overexpressed, and related to precocious dementia.
|
