Clinical Characteristics of Children with Rett Syndrome

Zee-A Han,Ha Ra Jeon,김성우,박진영,Hee Jung Chung 대한재활의학회 2012 Annals of Rehabilitation Medicine Vol.36 No.3

Objective To identify the clinical characteristics and investigate function related aspects of Korean children with Rett syndrome. Method A total of 26 patients diagnosed as Rett syndrome were clinically observed until the age of fi ve or over. We surveyed past history, developmental history, and presence of typical clinical features of Rett syndrome. Furthermore, we investigated diff erences in clinical characteristics according to functional status and changes in clinical features related to growth. Results There were no problems related to gestational, perinatal or neonatal history. Only 12 patients had an ultimate head circumference of less than 3 percentile. Developmental regression was definite in all patients. At final assessment, only 14 patients were able to walk. Twenty patients had an epileptic history requiring medication. Sixteen patients with scoliosis showed progression during serial follow-up. Th e percentage of patients who were able to walk before 16 months was higher in the high function group than the low function group. Th e age of regression was 5.4 and 4.0 years in the high and low function group respectively, but the diff erence was not statistically signifi cant. Scoliosis was more severe and seizure onset age was younger in the low function group. Conclusion We investigated 26 clinical characteristics in Korean children with Rett syndrome. Their clinical features change according to age, and we believe such knowledge could be utilized in rehabilitation to minimize their disabilities.

Single-Molecule Surface-Enhanced Raman Scattering as a Probe of Single-Molecule Surface Reactions: Promises and Current Challenges

Choi, Han-Kyu,Lee, Kang Sup,Shin, Hyun-Hang,Koo, Ja-Jung,Yeon, Gyu Jin,Kim, Zee Hwan American Chemical Society 2019 Accounts of chemical research Vol.52 No.11

<P><B>Conspectus</B></P><P>The initial observations of surface-enhanced Raman scattering (SERS) from individual molecules (single-molecule SERS, SMSERS) have triggered ever more detailed mechanistic studies on the SERS process. The studies not only reveal the existence of extremely enhanced and confined fields at the gaps of Ag or Au nanoparticles but also reveal that the spatial, spectral, and temporal behaviors of the SMSERS signal critically depend on many factors, including plasmon resonances of nanostructures, diffusion (lateral and orientational) of molecules, molecular electronic resonances, and metal-molecule charge transfers. SMSERS spectra, with their <I>molecular vibrational fingerprints</I>, should in principle provide molecule-specific information on individual molecules in a way that any other existing single-molecule detection method (such as the ones based on fluorescence, mechanical forces, or electrical currents) cannot. Therefore, by following the spectro-temporal evolution of SMSERS signals of reacting molecules, one should be able to follow chemical reaction events of individual molecules without any additional labels. Despite such potential, however, real applications of SMSERS for single-molecule chemistry and analytical chemistry are scarce.</P><P>In this Account, we discuss whether and how we can use SMSERS to monitor single-molecule chemical kinetics. The central problem lies in the experimental challenges of separately characterizing and controlling various sources of fluctuations and spatial variations in such a way that we can extract only the chemically relevant information from time-varying SMSERS signals. This Account is organized as follows. First, we outline the standard theory of SMSERS, providing an essential guide for identifying sources of spatial heterogeneity and temporal fluctuations in SMSERS signals. Second, we show how single-molecule reaction events of surface-immobilized reactants manifest themselves in experimental SMSERS trajectories. Comparison of the reactive SMSERS data (magnitudes and frequencies of discrete transitions) and the predictions of SMSERS models also allow us to assess how faithfully the SMSERS models represent reality. Third, we show how SMSERS spectral features can be used to discover new reaction intermediates and to interrogate metal-molecule electronic interactions. Finally, we propose possible improvements in experimental design (including nanogap structures and molecular systems) to make SMSERS applicable to a broader range of chemical reactions occurring under ambient conditions. The specific examples discussed in this Account are centered around the single-molecule photochemistry of 4-nitrobenzenethiol on metals, but the conclusions drawn from each example are generally applicable to any reaction system involving small organic molecules.</P> [FIG OMISSION]</BR>

Wavelet 변환 계수를 이용한 대용량 지형정보 데이터의 삼각형 메쉬근사에 관한 연구

유한주(Han Ju Yu),이상지(Sang Zee Lee),나종범(Jong Beom Ra) 한국방송·미디어공학회 1997 방송공학회논문지 Vol.2 No.1

웨이브렛 변환을 이용한 지형정보의 고속 메쉬 근사기법

유한주(Han Ju Yu),이상지(Sang Zee Lee),나종범(Jong Beom Ra) 대한전자공학회 1997 대한전자공학회 학술대회 Vol.1997 No.6

A novel germ cell-specific protein, SHIP1, forms a complex with chromatin remodeling activity during spermatogenesis.

Choi, Eunyoung,Han, Cecil,Park, Inju,Lee, Boyeon,Jin, Sora,Choi, Heejin,Kim, Do Han,Park, Zee Yong,Eddy, Edward M,Cho, Chunghee American Society for Biochemistry and Molecular Bi 2008 The Journal of biological chemistry Vol.283 No.50

<P>To determine the mechanisms of spermatogenesis, it is essential to identify and characterize germ cell-specific genes. Here we describe a protein encoded by a novel germ cell-specific gene, Mm.290718/ZFP541, identified from the mouse spermatocyte UniGene library. The protein contains specific motifs and domains potentially involved in DNA binding and chromatin reorganization. An antibody against Mm.290718/ZFP541 revealed the existence of the protein in testicular spermatogenic cells (159 kDa) but not testicular and mature sperm. Immunostaining analysis of cells at various stages of spermatogenesis consistently showed that the protein is present in spermatocytes and round spermatids only. Transfection assays and immunofluorescence studies indicate that the protein is localized specifically in the nucleus. Proteomic analyses performed to explore the functional characteristics of Mm.290718/ZFP541 showed that the protein forms a unique complex. Other major components of the complex included histone deacetylase 1 (HDAC1) and heat-shock protein A2. Disappearance of Mm.290718/ZFP541 was highly correlated with hyperacetylation in spermatids during spermatogenesis, and specific domains of the protein were involved in the regulation of interactions and nuclear localization of HDAC1. Furthermore, we found that premature hyperacetylation, induced by an HDAC inhibitor, is associated with an alteration in the integrity of Mm.290718/ZFP541 in spermatogenic cells. Our results collectively suggest that the Mm.290718/ZFP541 complex is implicated in chromatin remodeling during spermatogenesis, and we provide further information on the previously unknown molecular mechanism. Consequently, we re-designate Mm.290718/ZFP541 as 'SHIP1' representing spermatogenic cell HDAC-interacting protein 1.</P>

Factors Related to the Occurrence of Urinary Tract Infection Following a Urodynamic Study in Patients With Spinal Cord Injury

황성일,Bum-Suk Lee,Zee-A Han,Hye-Jin Lee,Sang-Hoon Han,Myeong-Ok Kim 대한재활의학회 2016 Annals of Rehabilitation Medicine Vol.40 No.4

Objective To analyze the factors related to urinary tract infection (UTI) occurrence after an urodynamic study (UDS) in patients with spinal cord injury (SCI).Methods We retrospectively investigated the medical records of 387 patients with SCI who underwent UDS with prophylactic antibiotic therapy between January 2012 and December 2012. Among them, 140 patients met the inclusion criteria and were divided into two groups, UTI and non-UTI. We statistically analyzed the following factors between the two groups: age, sex, level of injury, SCI duration, spinal cord independence measure, non-steroidal anti-inflammatory drug use, diabetes mellitus, the American Spinal Injury Association impairment scale (AIS), lower extremity spasticity, a history of UTI within the past 4 weeks prior to the UDS, symptoms and signs of neurogenic bladder, urination methods, symptoms during the UDS and UDS results. Results Among the 140 study participants, the UTI group comprised 12 patients and the non-UTI group comprised 128 patients. On univariate analysis, a history of UTI within the past 4 weeks prior to the UDS was significant and previous autonomic dysreflexia before the UDS showed a greater tendency to influence the UTI group. Multivariable logistic regression analysis using these two variables showed that the former variable was significantly associated with UTI and the latter variable was not significantly associated with UTI.Conclusion In patients with SCI, a history of UTI within the past 4 weeks prior to the UDS was a risk factor for UTI after the UDS accompanied by prophylactic antibiotic therapy. Therefore, more careful pre-treatment should be considered when these patients undergo a UDS.

Protective Role of Tissue Transglutaminase in the Cell Death Induced by TNF-α in SH-SY5Y Neuroblastoma Cells

Kweon, Soo-Mi,Lee, Zee-Won,Yi, Sun-Ju,Kim, Young-Myeong,Han, Jeong-A,Paik, Sang-Gi,Ha, Kwon-Soo Korean Society for Biochemistry and Molecular Biol 2004 Journal of biochemistry and molecular biology Vol.37 No.2

Tissue transglutaminase (tTGase) regulates various biological processes, including extracellular matrix organization, cellular differentiation, and apoptosis. Here we report the protective role of tTGase in the cell death that is induced by the tumor necrosis factor $\alpha$ (TNF-$\alpha$) and ceramide, a product of the TNF-$\alpha$ signaling pathway, in human neuroblastoma SH-SY5Y cells. Treatment with retinoic acid (RA) induced the differentiation of the neuroblastoma cells with the formation of extended neurites. Immunostaining and Western blot analysis showed the tTGase expression by RA treatment. TNF-$\alpha$ or $C_2$ ceramide, a cell permeable ceramide analog, induced cell death in normal cells, but cell death was largely inhibited by the RA treatment. The inhibition of tTGase by the tTGase inhibitors, monodansylcadaverine and cystamine, eliminated the protective role of RA-treatment in the cell death that is caused by TNF-$\alpha$ or $C_2$-ceramide. In addition, the co-treatment of TNF-$\alpha$ and cycloheximide ecreased the protein level of tTGase and cell viability in the RA-treated cells, supporting the role of tTGase in the protection of cell death. DNA fragmentation was also induced by the co-treatment of TNF-$\alpha$ and cycloheximide. These results suggest that tTGase expressed by RA treatment plays an important role in the protection of cell death caused by TNF-$\alpha$ and ceramide.

Coumarin-Cu(II) ensemble-based cyanide sensing chemodosimeter.

Jung, Hyo Sung,Han, Ji Hye,Kim, Zee Hwan,Kang, Chulhun,Kim, Jong Seung American Chemical Society 2011 ORGANIC LETTERS Vol.13 No.19

<P>An 'ensemble'-based chemodosimeter 1-Cu(II) for cyanide detection is reported. 1-Cu(II) can recognize a cyanide ion over other anionic species to show a marked fluorescence enhancement under aqueous conditions. 'Off-on' fluorescence change of 1-Cu(II) is proceeded by addition of cyanide, which induces decomplexation of the Cu(II) ion from nonfluorescent 1 followed by hydrolytic cleavage of the resulted Schiff base to give a strongly fluorescent coumarinaldehyde (2). The selective detection of cyanide with 1-Cu(II) for biological application was also performed in HepG2 cells.</P>

Neurodevelopmental Disorders of Children Screened by The Infantile Health Promotion System

김성우,Ha Ra Jeon,Zee-A Han,최자영,Hee Jung Chung,Young Key Kim,Yeo Hoon Yoon 대한재활의학회 2011 Annals of Rehabilitation Medicine Vol.35 No.6

Objective To perform an in depth evaluation of children, and thus provide a systematic method of managing children, who after infantile health screening, were categorized as suspected developmental delay. Method 78 children referred to the Developmental Delay Clinic of Ilsan Hospital after suspected development delay on infantile health examinations were enrolled. A team comprised of a physiatrist, pediatrician and pediatric psychiatrist examined the patients. Neurological examination, speech and cognitive evaluation were done. Hearing tests and chromosome studies were performed when needed clinically. All referred children completed K-ASQ questionnaires. Final diagnoses were categorized into specific language impairment (SLI),global developmental delay (GDD), intellectual disability (ID), cerebral palsy (CP), motor developmental delay (MD) or autism spectrum disorder (ASD). Results 72 of the 78 patients were abnormal in the final diagnosis, with a positive predictive value of 92.3%. Thirty (38.4%) of the 78 subjects were diagnosed as GDD, 28 (35.8%) as SLI, 5 (6.4%) as ASD, 9 (12.5%) as MD, and 6(7.6%) as normal. Forty five of the 78 patients had risk factors related to development, and 18 had a positive family history for developmental delay and/or autistic disorders. The mean number of abnormal domains on the K-ASQ questionnaires were 3.6 for ASD, 2.7 for GDD, 1.8 for SLI and 0.6 for MD. Differences between these numbers were statistically significant (p<0.05). Conclusion Because of the high predictive value of the K-ASQ, a detailed evaluation is necessary for children suspected of developmental delay in an infantile health promotion system.

뇌졸중환자에서 뇌지도화를 통해 증명된 동측 운동신경 경로

장성호,한봉수,장용민,변우목,이지인,이호 한국뇌학회 2001 한국뇌학회지 Vol.1 No.2

목적: 뇌졸중환자에서 fMRI와 TMS를 이용하여 수부의 운동신경기능에 대한 뇌지도화를 시행하여 운동신경기능 회복 기전에 대하여 연구하고자 하였다. 방법: 40세 여자 환자로 우측 심부 백질 경색으로 인한 좌측 편마비환자이었다. 기능적 자기공명영상은 1.5T MR scanner로 Blood Oxygen Level-Dependent(BOLD) 기법을 적용하였다. 운동 과제는 손가락을 1∼2 Hz의 주기로 쥐었다 펴기를 반복하였다. TMS는 원형코일의 앞쪽 부위를 1.0 cm 간격으로 자극하여 양측 단무지외전근에서 운동유발전위를 얻었다. 결과: FMRI를 시행한 결과 건측인 우측 수부운동 시 좌측 일차 감각운동피질(SM1)이 활성화되었다. 환측인 좌측 수부운동 시에는 양측 SM1이 활성화되었다. TMS를 이용한 뇌지도화에서는 건측인 좌측 대뇌 피질로부터 환측인 좌측 상지로 가는 동측 운동유발전위가 유발되었다. 동측 운동유발전위는 좌측 대뇌 피질을 자극하여 우측 단무지외전근에서 유발된 운동유발전위에 비하여 잠시가 지연되어 있고 전위가 감소되어 있었다. 결론: FMRI와 TMS를 이용한 뇌지도화를 통하여 건측 대뇌피질로부터 환측 상지로의 동측 운동신경 경로에 의하여 운동신경기능 회복이 되었으며 이 동측 운동신경 경로는 비피질척수로에서 기인된 것으로 추정할 수 있었다. Objective: This study investigated the mechanism of motor recovery using both functional Magnetic Resonance Imaging(fMRI) and Transcranial Magnetic Stimulation(TMS) in a left hemiplegic patient with infarction on the right deep white matter. Method: FMRI was performed using blood oxygen level-dependent(BOLD) technique at 1.5 T with a standard head coil. The motor activation task consisted of finger flexion-extension exercises in 1-2 Hz cycles. TMS was carried out using a round coil. The anterior portion of the coil was moved over different scalp positions 1.0 cm apart. Motor evoked potential(MEP) from both abductor pollicis brevis(APB) muscle was obtained simultaneously Results: FMRI showed that the left primary sensorimotor cortex(SM1) was activated with the right hand movements. On the other hand, the bilateral SM1 were activated with the left hand movements. Brain mapping using TMS revealed that ipsilateral MEPs were obtained at the left APB muscle. Ipsilateral MEPs of left APB muscle showed delayed latency and lower amplitude compared to that of right APB muscle when stimulated at the left motor cortex. Conclusions: We concluded that ipsilateral motor pathway from undamaged motor cortex seemed to contribute to the motor recovery in this patient. The ipsilateral motor pathway was considered to be originated from non-corticospinal tract by its configuration.