• Development and Reproduction
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• v.12 no.1
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• pp.1-8
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• 2008

Specific protocols to increase the differentiation of neuronal cells from embryonic stem (ES) cells have been well established, such as retinoic acid induction and lineage selection of neuronal cells. For the neuropathological studies, ES-derived neurons (ES neurons) must show normal physiological characteristics related to cell death and survival and should be maintained in vitro for a sufficient time to show insults-specific cell death without spontaneous death. When mouse ES cells were plated onto astrocytes monolayer after retinoic acid induction, most ES cells differentiated into neuronal cells, which were confirmed by the presence of specific neuronal markers, and the cultures were viable for at least four weeks. When these cultures were examined for vulnerability to glutamate excitotoxicity, ES neurons were vulnerable to excitotoxic insults mediated by agonist-specific receptors. The vulnerability to excitotoxic death increased with developmental age of ES neurons in vitro. Specific receptors for Neurotrophin and GDNF family ligands were present in ES neurons. GDNF and NT-3 could modulate the survival and excitotoxic vulnerability of ES neurons. The vulnerability and resistance to toxic insults, which are essential requirements of model culture systems for neuropathological studies, make ES neurons to a useful model culture system. Especially ES cell are highly amenable to genetic modification unlikely to primary neuronal cells, which will give us a chance to answer more complicated neurophysiological questions. Recently there was an outstanding attempt to explore the cellular toxicity using human ES cells (Schrattenholz & Klemm, 2007) and it suggested that ES cells could be a new model system for neurophysiological studies soon and go further a large-scale screening system for pharmacological compounds in the future.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.924-928
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• 2005

Purpose : Treatment efficacy for children with speech and language delay has been the subject of considerable debate in recent years. We evaluated the clinical features of children with delayed speech and language and their prognoses according to their etiologies after 6 months of speech and language therapy. Methods : From January, 2000 to March, 2004, we retrospectively reviewed 56 children with speech and language delay who were administered speech and language therapy for 6 months in Uijongbu St. Mary's Hospital. Results : Of 56 cases, the proportion of developmental language disorder was 66.1 percent, structural malformation 19.6 percent, mental retardation 12.5 percent, hearing defect 1.8 percent. The ratio of male to female was 4.6 : 1 and the most frequent age group was over 47 months. The mean age of first spontaneous words with useful meaning was 15.9 months. The mean gestational age of the subjects was 39.8 weeks. The proportion of full-term infants was 96.4 percent and of premature infants was 3.6 percent. As for the birth order, the proportion of the first baby was 51.8 percent, the one of second babies it was 42.9 percent, and percent of third babies it was 7.1 percent. After 6 months of language intervention, 32.4 percent of patients with developmental language disorder showed normal linguistic development. All the patients with mental retardation showed sustained language and speech delay. As for the patients with structural malformations, five out of 11 patients showed normal linguistic development. Conclusion : The relatively advanced old age of majority of participants in this study suggests the necessity of screening test for language delay in this local community.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.964-970
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• 2008

Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.

• Development and Reproduction
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• v.12 no.3
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• pp.289-296
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• 2008

BPES (Blepharophimosis/Ptosis/Epicanthus inversus Syndrome) is an autosomal dominant disorder caused by mutations in FOXL2. Affected individuals have premature ovarian failure (POF) in addition to small palpebral fissures, drooping eyelids, and broad nasal bridge. FOXL2 is a member of the forkhead family transcription factors. In FOXL2-deficient ovaries, granulosa cell differentiation dose not progress, leading to arrest of folliculogenesis and oocytes atresia. Using yeast two-hybrid screening of rat ovarian cDNA library with FOXL2 as bait, we found that small ubiquitin-related modifier (SUMO)-conjugating E2 enzyme UBE2I protein interacted with FOXL2 protein. UBE2I also known as UBC9 is an essential protein for processing SUMO modification. Sumoylation is a form of post-translational modification involved in diverse signaling pathways including the regulation of transcriptional activities of many transcriptional factors. In the present study, we confirmed the protein-protein interaction between FOXL2 and UBE2I in human cells, 293T, by in vivo immunoprecipitation. In addition, we generated truncated FOXL2 mutants and identified the region of FOXL2 required for its association with UBE2I using yeast-two hybrid system. Therefore, the identification of UBE2I as an interacting protein of FOXL2 further suggests a presence of novel regulatory mechanism of FOXL2 by sumoylation.

• Korean Journal of Psychosomatic Medicine
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• v.19 no.2
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• pp.74-82
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• 2011

Objective:The aim of this study was to evaluate the relationship between sustained attention deficits in Attention-Deficit/Hyperactivity Disorder(ADHD) children and short-term Heart Rate Variability(HRV) parameters. In addition, we evaluate the relationship between The ADHD rating scale(ARS), the computerized ADHD diagnostic system(ADS) and Parenting stress index- short form(PSI-SF). Methods:This study was performed in the department of children and Adolescent psychiatry, Korea university Guro hospital from august 2008 to January 2009. We evaluated HRV parameters by short-term recordings of 5 minutes. K-ARS and ADS are used for screening and identifying ADHD children. Intelligence was measured using Korean educational Developmental Institute-wechsler Intelligence Scale for Children. The caregivers Complete Parenting Stress Index scale for evaluation parent stress. Results:The low frequency(LF) was significantly correlated with response variability of ADS. However, the other variables of ARS and ADS were not significantly correlated with LF. Hyperactivity subscale of ARS was significantly correlated with parental distress subscale and difficult child subscale of PSI-SF and inattention subscale of ARS was also significantly correlated with dysfunctional interaction and difficult child subscale of PSI-SF. Conclusion:The LF, 0.10-Hz component of HRV is known to measure effort allocation. This study shows that the LF component of HRV is significantly correlated with the response variability of ADS. This means that more severe symptoms of ADHD were correlated with the increase in the LF that means decreased effort allocation. These results also support the clinical usability of HRV in the assessment of ADHD. Furthermore, PSI-SF is correlated with hyperactivity and inattention variables of ARS.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.36 no.3
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• pp.214-219
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• 1991

To clarify the relationship between ambient water temperature and heading characteristics of rice (Oryza sativa L.), twelve of varieties rice were grown in a cold tolerance screening field where water temperature was controlled by continuous cold water irrigation system to test the cold tolerance of rice. When cold water was continuously irrigated with 5 cm of water depth, the water had stagnated for about three hours, and the water temperature increased gradually from inlet toward outlet in the experimental plot. The fluctuation of water temperature was well synchronized with that of ambient air temperature, and the water temperature near outlet became higher than the air temperature at the vegetative phase, while became lower at the reproductive phase of rice plant community. The leaf development rates on main culm increased by increased water temperature. The rice varities, Fukuhikari, Sangpungbyeo and YR3486-16-2 were more sensitive than the others in the response of leaf development to water temperature. However, Janack and Milyang 42 were comparatively less sensitive to water temperature in leaf development. Janack and Paro -white rices required longer days to develop one leaf on main culm at reproductive phase than at vegetative phase. Varietal difference in days required to develop one leaf on main culm of rice plant was more distinctive at the reproductive phase when water temperature was relatively lower than at the vegetative phase with relatively higher water temperature condition. No difference was found between the growth phases, vegetative and reproductive, in the response of average leaf developmental rates to water temperature under the similar air temperature condition. The estimated average days required to develop one leaf on main culm decreased by 1.3 day by 1$^{\circ}C$ increase in water temperature. Varietal differences in the total number of leaves on main culm depended upon the water temperature, in which the varieties such as Fukuhikari, Gwangmyeon-gbyeo, China 988, and YR3486-16-2 showed increased one leaf by increased water temperature, while Sobaekbyeo, Paro-white, Sangpungbyeo, Pungsanbyeo, Samgangbyeo, and Milyang 42 were kept at the same leaf number regardless of water temperature. However, the total leaf number on main culm and days to heading of Janack increased by increased water temperature. The other varieties showed the shortened days to heading by the increase in water temperature with noticeable varietal differences regardless of the variation in the total number of leaves on main culm.

• Development and Reproduction
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• v.13 no.1
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• pp.1-11
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• 2009

Implantation itself is governed by an array of endocrine, paracrine and autocrine modulators, of embryonic and maternal origin. Window of implantation is the unique temporal and spatial expression of factors allows the embryo to implant via signaling, appositioning, attachment, and invasion in a specific time frame of $2{\sim}4$ days. When the embryo has arrived in the uterine cavity, a preprogrammed sequence of events occurs, which involves the production and secretion of a multitude of biochemical factors such as cytokines, growth factors, and adhesion molecules by the endometrium and the embryo, thus leading to the formation of a receptive endometrium. Cytokines such as LIF, CSF-1, and IL-1 have all been shown to play important roles in the cascade of events that leads to implantation. Integrin, L-selectin ligands, glycodelin, mucin-1, HB-EGF and pinopodes are involved in appositioning and attachment. The embryo also produces cytokines and growth factors (ILs, VEGF) and receptors for endometrial signals such as LIF, CSF-1, IGF and HB-EGF. The immune system and angiogenesis play an important role. The usefulness of these factors to assess endometrial receptivity and to estimate the prognosis for pregnancy in natural and artificial cycles remains to be proven. Integrins, pinopodes, glycodelin and LIF (from biopsies) are promising candidates; from uterine flushings, glycodelin and LIF are also candidates. The ideal serum marker is not available, but VEGF, glycodelin and CSF have some clinical implications. Further evaluation that includes larger groups of infertile women and fertile controls are needed to elucidate whether their presence in plasma, flushing fluid, or endometrial samples can be used as some kind of a screening tool to assess endometrial function and prognosis for pregnancy before and after artificial reproductive therapy. A better understanding of their function in human implantation may lead to therapeutic intervention, thereby improving the success rate in reproduction treatment. New molecular techniques are becoming available for measuring both embryonic and endometrial changes prior to and during implantation. The use of predictive sets of markers may prove to be more reliable than a single marker. Ultimately, the aim is to use these tools to increase implantation in artificial cycles and consequently improve live-birth rates.