• BMB Reports
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• v.51 no.7
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• pp.356-361
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• 2018

Actin-binding LIM protein 1 (ABLIM1), a member of the LIM-domain protein family, mediates interactions between actin filaments and cytoplasmic targets. However, the role of ABLIM1 in osteoclast and bone metabolism has not been reported. In the present study, we investigated the role of ABLIM1 in the receptor activator of $NF-{\kappa}B$ ligand (RANKL)-mediated osteoclastogenesis. ABLIM1 expression was induced by RANKL treatment and knockdown of ABLIM1 by retrovirus infection containing Ablim1-specific short hairpin RNA (shAblim1) decreased mature osteoclast formation and bone resorption activity in a RANKL-dose dependent manner. Coincident with the downregulated expression of osteoclast differentiation marker genes, the expression levels of c-Fos and the nuclear factor of activated T-cells cytoplasmic 1 (NFATc1), critical transcription factors of osteoclastogenesis, were also decreased in shAblim1-infected osteoclasts during RANKL-mediated osteoclast differentiation. In addition, the motility of preosteoclast was reduced by ABLIM1 knockdown via modulation of the phosphatidylinositol-4,5-bisphosphate 3-kinase (PI3K)/Akt/Rac1 signaling pathway, suggesting another regulatory mechanism of ABLIM1 in osteoclast formation. These data demonstrated that ABLIM1 is a positive regulator of RANKL-mediated osteoclast formation via the modulation of the differentiation and PI3K/Akt/Rac1-dependent motility.

• Journal of Korean Academy of Nursing
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• v.29 no.3
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• pp.467-484
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• 1999

The main objectives of this study were to investigate the concept of family function from the perspective of the contemporary Korean family, and to construct model of change of family function with chronic illness. The hybrid model approach was applied in which three phases(theoretical phase, empirical phase, and analytic phase) of concept development were explored for family functioning. The study was conducted from 1997 to 1998. In empirical phase, two groups of purposive samples were drawn : normal family group composed of six families without ill family member, and ill family group composed of seven families of which wives have rheumatoid arthritis. Only families with child(or children) in primary or secondary schools were included in the study. The results were as follows : In theoretical phase, six dimensions of family concept were emerged : affective, structural, control, cognitive, financial, and reproductive dimension. In order to analyse the Korean normal family function in middle class with middle-aged women, financial and reproductive dimension were not included. In empirical phase, five dimensions(affective, structural, control, cognitive, and external relationship) were found from the normal family data. External relationship dimension is very important factor as a resource of the support, especially when their parents or siblings had no help or support to them. In the affective dimension, Korean family emphasized harmony and balance rather than affective expression between couples and between parents and children. They also showed common goals of the families to solve their problems to control the family members. The priority of the goals was getting into the higher education of their children or helping their unhealthy parents or family members. Six dimensions (affective, structural, control, cognitive, external relationship, and financial) of family functions were emerged from the ill family data. From the analysis of ill family data, types of restructuring house chore after wives illness were developed : (a) negociated, (b) accomodated, and (c) isolated, enduring types. Although the dimensions of family functioning identified in this study are similar to the conceptualizations that exist in the western literature, there were distinct differences in the nature of major themes and subconcepts under these family function dimensions.

• Journal of Genetic Medicine
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• v.21 no.1
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• pp.31-35
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• 2024

Allan-Herndon-Dudley syndrome (AHDS) is a rare X-linked neurodevelopmental disorder with abnormal thyroid function caused by mutation in the solute carrier family 16 member 2 (SLC16A2) gene. Clinical manifestations of AHDS are global or axial hypotonia, a variety of movement disorders, severe intellectual disability, quadriplegia or spastic diplegia, growth failure, and seizures. A 10-year-old boy visited our hospital with the chief complaint of newly onset generalized tonic seizures with vocalization of weekly to daily frequency. He showed early infantile hypotonia, severe intellectual disability, and frequent respiratory infections. He could not walk independently and was non-verbal. Electroencephalogram revealed generalized slow spike and waves with multifocal spikes and slow background rhythms. His tonic seizures were controlled with more than two anti-seizure medications (ASMs). At 11 years of age, he was evaluated for thyroid function as part of regular screening for ASM maintenance and was found to have abnormal thyroid function. We performed whole exome sequencing for severe global developmental delay, drug-resistant epilepsy, and abnormal thyroid function. The hemizygous c.940C>T (p.Arg314Ter) variant in the SLC16A2 gene (NM_006517.5) was identified and confirmed based on Sanger sequencing. Herein, we describe a case of an AHDS patient with late-onset drug-resistant epilepsy combined with congenital hypotonia, global developmental delay, and abnormal thyroid function results. To the best of our knowledge, this is the oldest adolescent among AHDS cases reported in Korea. In this report, clinical characteristics of a mid-adolescence patient with AHDS were presented.

• Korean Journal of Construction Engineering and Management
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• v.12 no.5
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• pp.12-22
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• 2011

Design value engineering (VE) has been performed in many construction projects according to the changes in the construction for most of the VB function analysis that performed, are problems that focus on function analysis and generating ideas based on individual experience of VE team member than generating ideas and value improvement of systematic function analysis. This may be due to the quotation of previously performed function analysis data, general function analysis, and abstract function definitions that do not cite the concrete characteristics of each facility. Therefore, in this study, the proposed cases from the existing design VE workshops, the current conditions of the connection between functions and ideas and of the idea categorization by building part were studied to determine why functions and ideas are not connected, and to propose pertinent improvement directions. on a performance-based function categorization system by building part that can provide the logical connection between functions and ideas in the function definition/categorization and function arrangement phases in the function analysis phase, the most important phase in VE activities, was developed by reflecting the required performance and functions for each part of the facility.

• Journal of Microbiology and Biotechnology
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• v.11 no.4
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• pp.543-551
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• 2001

The vascular endothelial growth factor (VEGF), or VEGF-A, is intimately involved in both physiological and pathological forms of angiogenesis. VEGF-A is now recognized as the founding member of a family of growth factors that has expanded to include VEGF-B, VEGF-C, VEGF-D, VEGF-E, and placental growth factor (PIGF). This family of cytokines binds differentially to at least three receptor tyrosine kinases, however, the extent to which family members other than VEGF-A contribute to physiological and pathological angiogenesis remains unclear. Issues that are of relevance include uncertainty regarding the consequences of signaling through VEGF - RI in particular, and the ability of some family members to heterodimerize, leading to the possibility ofheterodimeric receptor complexes. Structural characterization is one approach that can be used to address these issues, however, the vast majority of previous structure-function studies have only focused on VEGF-A. While these studies may provide some clues regarding the structural basis of the interaction of other family members with their receptors, studies using the ligands themselves are clearly required if highly specific interactions are to be revealed. With the recent progress toward refolding and purifying substantial' quantities of other VEGF family members, such structural studies are now possible. Here, these ~ssues are addressed with a particular emphasis on VEGF-B and its receptors.

• Earthquakes and Structures
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• v.1 no.1
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• pp.43-67
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• 2010

A gradient-based evolutionary optimization methodology is presented for finding the optimal design of both the added dampers and their supporting members to minimize an objective function of a linear multi-storey structure subjected to the critical ground acceleration. The objective function is taken as the sum of the stochastic interstorey drifts. A frequency-dependent viscoelastic damper and the supporting member are treated as a vibration control device. Due to the added stiffness by the supplemental viscoelastic damper, the variable critical excitation needs to be updated simultaneously within the evolutionary phase of the optimal damper placement. Two different models of the entire damper unit are investigated. The first model is a detailed model referred to as "the 3N model" where the relative displacement in each component (i.e., the spring and the dashpot) of the damper unit is defined. The second model is a simpler model referred to as "the N model" where the entire damper unit is converted into an equivalent frequency-dependent Kelvin-Voigt model. Numerical analyses for 3 and 10-storey building models are conducted to investigate the characters of the optimal design using these models and to examine the validity of the proposed technique.

• Steel and Composite Structures
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• v.16 no.2
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• pp.135-156
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• 2014

In this research the effect of seismic design level as a practical approach for progressive collapse mitigation and reaching desired structural safety against it in seismically designed concentric braced frame buildings was investigated. It was achieved by performing preliminary and advanced progressive collapse analysis of several split-X braced frame buildings, designed for each seismic zone according to UBC 97 and by applying various Seismic Load Factors (SLFs). The outer frames of such structures were studied for collapse progression while losing one column and connected brace in the first story. Preliminary analysis results showed the necessity of performing advanced element loss analysis, consisting of Vertical Incremental Dynamic Analysis (VIDA) and Performance-Based Analysis (PBA), in order to compute the progressive collapse safety of the structures while increasing SLF for each seismic zone. In addition, by sensitivity analysis it became possible to introduce the equation of structural safety against progressive collapse for concentrically braced frames as a function of SLF for each seismic zone. Finally, the equation of progressive collapse safety as a function of bracing member capacity was presented.

• KIPS Transactions on Software and Data Engineering
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• v.2 no.11
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• pp.739-746
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• 2013

Variability of the software product line that differentiates member products within a product line must be described with precise meaning and visualized so as easy to select. Moreover, it should be easy to manage. Variability description approaches can largely be divided into two approaches, integrated variability description approach and orthogonal variability description approach. Orthogonal Variability Description Language (OVDL) was developed for clear and precise description of variability without ambiguity. This paper validates the variability description capability of OVDL by translating the variability models of Inter-Working Function (IWF) product line described by using Orthogonal Variability Model (OVM) notations into variability descriptions in OVDL.

• BMB Reports
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• v.48 no.2
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• pp.97-102
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• 2015

Neurons in the developing brain form the cortical plate (CP) in an inside-out manner, in which the late-born neurons are located more superficially than the early-born neurons. Fyn, a member of the Src family kinases, plays an important role in neuronal migration by binding to many substrates. However, the role of the Src-homology 2 (SH2) domain in function of Fyn in neuronal migration remains poorly understood. Here, we demonstrate that the SH2 domain is essential for the action of Fyn in neuronal migration and cortical lamination. A point mutation in the Fyn SH2 domain ($Fyn^{R176A}$) impaired neuronal migration and their final location in the cerebral cortex, by inducing neuronal aggregation and branching. Thus, we provide the first evidence of the Fyn SH2 domain contributing to neuronal migration and neuronal morphogenesis.

• Earthquakes and Structures
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• v.11 no.4
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• pp.701-721
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• 2016

Base isolation is a well-established passive strategy for seismic response control of buildings. In this paper, an efficient framework is proposed for reliability-based design optimization (RBDO) of isolated buildings subjected to uncertain earthquakes. The framework uses reduced function evaluations method, as an efficient tool for structural reliability analysis, and an efficient optimization algorithm for optimal structural design. The probability of failure is calculated considering excessive base displacement, superstructure inter-storey drifts, member stress ratios and absolute accelerations of floors of the isolated building as failure events. The behavior of rubber bearing isolators is modeled using nonlinear hysteretic model and the variability of future earthquakes is modeled by applying a probabilistic approach. The effects of pulse component of stochastic near-fault ground motions, fixity-factor of semi-rigid beam-to-column connections, values of isolator parameters, earthquake magnitude and epicentral distance on the performance and safety of semi-rigidly connected base-isolated steel framed buildings are studied. Suitable RBDO examples are solved to illustrate the results of investigations.