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Kim, Bong Jik,Ueyama, Takehiko,Miyoshi, Takushi,Lee, Seungmin,Han, Jin Hee,Park, Hye-Rim,Kim, Ah Reum,Oh, Jayoung,Kim, Min Young,Kang, Yong Seok,Oh, Doo Yi,Yun, Jiwon,Hwang, Sang Mee,Kim, Nayoung K D BMJ Publishing Group Ltd 2019 Journal of medical genetics Vol.56 No.12
<P><B>Background</B></P><P>Diaphanous-related formin 1 (DIA1), which assembles the unbranched actin microfilament and microtubule cytoskeleton, is encoded by <I>DIAPH1</I>. Constitutive activation by the disruption of autoinhibitory interactions between the N-terminal diaphanous inhibitory domain (DID) and C-terminal diaphanous autoregulatory domain (DAD) dysregulates DIA1, resulting in both hearing loss and blood cell abnormalities.</P><P><B>Methods and results</B></P><P>Here, we report the first constitutively active mutant in the DID (p.A265S) of humans with only hearing loss and not blood cell abnormality through whole exome sequencing. The previously reported DAD mutants and our DID mutant (p.A265S) shared the finding of diminished autoinhibitory interaction, abnormally upregulated actin polymerisation activity and increased localisations at the plasma membrane. However, the obvious defect in the DIA1-driven assembly of cytoskeleton ‘during cell division’ was only from the DAD mutants, not from p.A265S, which did not show any blood cell abnormality. We also evaluated the five DID mutants in the hydrophobic pocket since four of these five additional mutants were predicted to critically disrupt interaction between the DID and DAD. These additional pathogenic DID mutants revealed varying degrees of defect in the DIA1-driven cytoskeleton assembly, including nearly normal phenotype during cell division as well as obvious impaired autoinhibition, again coinciding with our key observation in DIA1 mutant (p.A265S) in the DID.</P><P><B>Conclusion</B></P><P>Here, we report the first mutant in the DID of humans with only hearing loss. The differential cell biological phenotypes of DIA1 during cell division appear to be potential determinants of the clinical severity of <I>DIAPH1-</I>related cytoskeletopathy in humans.</P>
The Level of SecA Synthesis During SecY , SecE , or SecY / SecE Overproduction in Escherichia coli
Kim, Jae Young,Lee, Sang Jik 생화학분자생물학회 1975 BMB Reports Vol.27 No.6
The level of SecA synthesis during Sect, SecE, or Sect/SecE overproduction was examined using pulse labeling techniques and β-galactosidase assays. The level of SecA synthesis increased during the early phase of induction of SecE or Sect/SecE, whereas it declined after induction of Sect. No defect in the processing of the maltose-binding protein or OmpA was detected under these conditions, implying that the increase in SecA synthesis was not in response to a defect in protein secretion under these conditions. These results suggest that secA may be regulated by the level of certain integral Sec membrane components with which it may interact.
Sang-Yeon Lee,Hyun Been Choi,Mina Park,Il Soon Choi,Jieun An,Ami Kim,Eunku Kim,Nahyun Kim,Jin Hee Han,Min young Kim,Seung min Lee,Doo-Yi Oh,Bong Jik Kim,Nayoung Yi,Nayoung, K. D. Kim,Chung Lee,Woong-Y 생화학분자생물학회 2021 Experimental and molecular medicine Vol.53 No.-
Loss-of-function variant in the gene encoding the KCNQ4 potassium channel causes autosomal dominant nonsyndromic hearing loss (DFNA2), and no effective pharmacotherapeutics have been developed to reverse channel activity impairment. Phosphatidylinositol 4,5-bisphosphate (PIP2), an obligatory phospholipid for maintaining KCNQ channel activity, confers differentialpharmacological sensitivity of channels to KCNQ openers. Through whole-exome sequencing of DFNA2 families, we identified three novel KCNQ4 variants related to diverse auditory phenotypes in the proximal C-terminus (p.Arg331Gln), the C-terminus of the S6 segment (p.Gly319Asp), and the pore region (p.Ala271_Asp272del). Potassium currents in HEK293T cells expressing each KCNQ4 variant were recorded by patch-clamp, and functional recovery by PIP2 expression or KCNQ openers was examined. In the homomeric expression setting, the three novel KCNQ4 mutant proteins lost conductance and were unresponsive to KCNQ openers or PIP2 expression. Loss of p.Arg331Gln conductance was slightly restored by a tandem concatemer channel (WT-p.R331Q), and increased PIP2 expression further increased the concatemer current to the level of the WT channel. Strikingly, an impaired homomeric p.Gly319Asp channel exhibited hyperactivity when a concatemer (WT-p.G319D), with a negative shift in the voltage dependence of activation. Correspondingly, a KCNQ inhibitor and chelation of PIP2 effectively downregulated the hyperactive WTp. G319D concatemer channel. Conversely, the pore-region variant (p.Ala271_Asp272del) was nonrescuable under any condition. Collectively, these novel KCNQ4 variants may constitute therapeutic targets that can be manipulated by the PIP2 level and KCNQregulating drugs under the physiological context of heterozygous expression. Our research contributes to the establishment of a genotype/mechanism-based therapeutic portfolio for DFNA2.
A Low Temperature Vacuum In-Line Sealing Technology for the High Performance PDP
Sang Jik Kwon,Jee-Hoon Kim 한국물리학회 2004 THE JOURNAL OF THE KOREAN PHYSICAL SOCIETY Vol.45 No.1
Sealing of the two glass plates comprising a plasma display panel (PDP) was done in a vacuum chamber by using an auxiliary heating line (AHL). In order to improve the uniformity of the sealing temperature and to reduce the panel temperature during sealing, we introduced an AHL under a frit glass by using a screen printing method, and we used it as part of the heating source for the frit melting. Although outgassing and bubbling can be severe problems during the heating process of frit glass inside a vacuum chamber, our specially prepared frit glass was successfully applied for vacuum in-line sealing without bubbles. During the sealing, the panel temperature was maintained at 250 C, and the local frit temperature was controlled at a sealing temperature around 350 C by applying a voltage to the AHL. By using the AHL technology and the specially prepared frit glass, we sealed an operational PDP panel with 2-inch-diagonal active area. The panel was successfully operated with continuous sustained 50-kHz pulses and gave a brilliant emission of light.
The Assessment of Remote Accessibility for Processing Equipment Using the Graphic Simulator
Sung-Hyun Kim,Hyo-Jik Lee,Seung-Nam Yu,Ki-Ho Kim,Il-je Cho 제어로봇시스템학회 2012 제어로봇시스템학회 국제학술대회 논문집 Vol.2012 No.10
In this paper, we describe a proposed assessment methodology through the analysis and evaluation of a graphic simulator as part of a remote accessibility. The processing equipment is operated in intense radiation fields as well as at high temperature. Thus, the equipment is operated in a remote manner and should be designed with consideration of remote handling and maintenance. This simulator is a virtual facility of a pyroprocessing mock-up facility to be constructed later. The simulator will be designed to include all the processing equipment, utilities, remote maintenance and handling devices and auxiliary devices or tools, in the form of virtual models which are necessary for the pyroprocessing technology implementation in a virtual facility environment. The proposed assessment methodology using remote accessibility and processing equipment of the 3D model was evaluated. The use of Using this device for operation and maintenance to assess the results is reflected in its modifications of the device.
Sang jik Kwon,Yong Jae Kim,Eou Sik Cho 한국물리학회 2007 THE JOURNAL OF THE KOREAN PHYSICAL SOCIETY Vol.50 No.I
MgO films were deposited with the various MgO evaporation rates and the physical characteristics of the MgO films were investigated and analyzed. The properties of MgO, such as crystal orientation, surface roughness, film structure, were inspected using X-ray diffraction (XRD), atomic force microscopy, and secondary electron microscopy. From the experiments and Paschen's law, the maximum value of the secondary electron emission coefficient ($\gamma$) was obtained at an evaporation rate of 5 \AA/sec. The XRD results and the cathodeluminescence spectra show that the $\gamma$ values are correlated with the F/F$^+$ centers of the molecule structures of the MgO films. The RMS surface roughness and the column-type structure also show the efficient characteristics of MgO film deposited at a rate of 5 \AA/sec.
Unit Generation Based on Phrase Break Strength and Pruning for Corpus-Based Text-to-Speech
Kim, Sang-Hun,Lee, Young-Jik,Hirose, Keikichi Electronics and Telecommunications Research Instit 2001 ETRI Journal Vol.23 No.4
This paper discusses two important issues of corpus-based synthesis: synthesis unit generation based on phrase break strength information and pruning redundant synthesis unit instances. First, the new sentence set for recording was designed to make an efficient synthesis database, reflecting the characteristics of the Korean language. To obtain prosodic context sensitive units, we graded major prosodic phrases into 5 distinctive levels according to pause length and then discriminated intra-word triphones using the levels. Using the synthesis unit with phrase break strength information, synthetic speech was generated and evaluated subjectively. Second, a new pruning method based on weighted vector quantization (WVQ) was proposed to eliminate redundant synthesis unit instances from the synthesis database. WVQ takes the relative importance of each instance into account when clustering similar instances using vector quantization (VQ) technique. The proposed method was compared with two conventional pruning methods through objective and subjective evaluations of synthetic speech quality: one to simply limit the maximum number of instances, and the other based on normal VQ-based clustering. For the same reduction rate of instance number, the proposed method showed the best performance. The synthetic speech with reduction rate 45% had almost no perceptible degradation as compared to the synthetic speech without instance reduction.