The First Korean Case of KCNQ2 Mutation in a Family with Benign Familial Neonatal Convulsions

Neonatal seizures represent a heterogeneous group of disorders with vastly different etiologies and outcomes. Benign familial neonatal convulsions (BFNC) are a distinctive epileptic syndrome of autosomal dominant inheritance with a favorable prognosis, characterized by the occurrence of unprovoked partial or generalized clonic seizures in the neonatal period or early infancy. Recently, mutations in two potassium channel genes, KCNQ2 and KCNQ3, have been described in this disorder. In this report, we describe a family with BFNC due to a KCNQ2 mutation, the first such family to be described in the Korean population. The diagnosis of BFNC can be made based on clinical suspicion and careful history taking with special emphasis on the familial nature of the disorder. KCNQ2 mutations may be associated with BFNC in a number of different races, as has been reported in other ethnic groups

Influx of Calcium through L-type Calcium Channels in Early Postnatal Regulation of Chloride Transporters in the Rat Hippocampus

During the early postnatal period, GABAB receptor activation facilitates L-type calcium current in rat hippocampus. One developmental process that L-type current may regulate is the change in expression of the K+Cl− co-transporter (KCC2) and N+K+2Cl− co-transporter (NKCC1), which are involved in the maturation of the GABAergic system. The present study investigated the connection between L-type current, GABAB receptors, and expression of chloride transporters during development. The facilitation of L-type current by GABAB receptors is more prominent in the second week of development, with the highest percentage of cells exhibiting facilitation in cultures isolated from 7 day old rats (37.5%). The protein levels of KCC2 and NKCC1 were investigated to determine the developmental timecourse of expression as well as expression following treatment with an L-type channel antagonist and a GABAB receptor agonist. The time course of both chloride transporters in culture mimics that seen in hippocampal tissue isolated from various ages. KCC2 levels increased drastically in the first two postnatal weeks while NKCC1 remained relatively stable, suggesting that the ratio of the chloride transporters is important in mediating the developmental change in chloride reversal potential. Treatment of cultures with the L-type antagonist nimodipine did not affect protein levels of NKCC1, but significantly decreased the upregulation of KCC2 during the first postnatal week. In addition, calcium current facilitation occurs slightly before the large increase in KCC2 expression. These results suggest that the expression of KCC2 is regulated by calcium influx through L-type channels in the early postnatal period in hippocampal neurons

STUDIES OF THE TACTILE PERCEPTION ON THE SYMNOETRICAL PARTS OF THE BODY. 3

departmental bulletin pape

STUDIES OF THE TACTILE PERCEPTION ON THE SYMMETRICAL PARTS OF THE BODY 2

departmental bulletin pape

Generating simplified ammonia reaction model using genetic algorithm and its integration into numerical combustion simulation of 1 MW test facility

A small reaction model for ammonia is necessary for the design and development of large-scale ammonia combustion systems using computational fluid dynamics (CFD) simulation. The present study generated a simplified reaction model for ammonia using a genetic algorithm (14 species and 45 reactions). Optimization was applied to reproduce the combustion properties of ignition delay times, mole fractions of ammonia and nitric oxide at the exit of a single perfectly stirred reactor (PSR), those of a two-staged PSR, and laminar flame speed. The generated simplified reaction model for ammonia was applied to combustion CFD simulation for a 1 MW boiler test facility. Results of CFD simulation reproduced the characteristics of two-staged combustion well. Nitrous oxide emissions predicted by the simulation agreed well with experimental results