The autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases, clinically and genetically heterogeneous, characterized by degeneration of spinocerebellar pathways with variable involvement of other neural systems. At present, 27 distinct genetic forms of SCAs are known: SCA1-8, SCA10-21, SCA23, SCA25-28, DRPLA (dentatorubral-pallidoluysian atrophy), and 16q-liked ADCA (autosomal dominant cerebellar ataxia). Epidemiological data about the prevalence of SCAs are restricted to a few studies of isolated geographical regions, and most do not reflect the real occurrence of the disease. In general a prevalence of about 0.3-2 cases per 100,000 people is assumed. As SCA are highly heterogeneous, the prevalence of specific subtypes varies between different ethnic and continental populations. Most recent data suggest that SCA3 is the commonest subtype worldwide; SCA1, SCA2, SCA6, SCA7, and SCA8 have a prevalence of over 2%, and the remaining SCAs are thought to be rare (prevalence <1%). In this review, we highlight and discuss the SCA7. The hallmark of SCA7 is the association of hereditary ataxia and visual loss caused by pigmentary macular degeneration. Visual failure is progressive, bilateral and symmetrical, and leads irreversibly to blindness. This association represents a distinct disease entity classified as autosomal dominant cerebellar ataxia (ADCA) type II by Harding. The disease affectsprimarily the cerebellum and the retina by the moderate to severe neuronal loss and gliosis, but also many other central nervous system structures as the disease progresses. SCA7 is caused by expansion of an unstable trinucleotide CAG repeat in the ATXN7 gene encoding a polyglutamine (polyQ) tract in the corresponding protein, ataxin-7. Normal ATXN7 alleles contain 4-35 CAG repeats, whereas pathological alleles contain from 36->450 CAG repeats. Immunoblott analysis demonstrated that ataxin-7 is widely expressed but that expression levels vary among tissues. Instability of expanded repeats is more pronounced in SCA7 than in other SCA subtypes and can cause substantial lowering of age at onset in successive generations termed ‘anticipation’ so that children may become diseased even before their parents develop symptoms. The strong anticipation in SCA7 and the rarity of contractions should have led to its extinction within a few generations. There is no specific drug therapy for this neurodegenerative disorder. Currently, therapy remains purely symptomatic. Cellular models and SCA7 transgenic mice have been generated which constitute valuable resources for studying the disease mechanism. Understanding the pathogenetic mechanisms of neurodegeneration in SCAs should lead to the identification of potential therapeutic targets and ultimately facilitate drug discovery. Here we summarize the clinical, pathological, and genetic aspects of SCA7, and review the current understanding of the pathogenesis of this disorder. Further, we also review the potential therapeutic strategies that are currently being explored in polyglutamine diseases.
Journal of the Korea Institute of Information and Communication Engineering
/
v.12
no.10
/
pp.1808-1814
/
2008
In addition to RBL function, which is used to applying for spam e-mail filtering, as an effective way to deal with the recently widespread spam types, this paper proposes how to extract URL that was comprised in the original e-mail, apply it to RBL, and expand it. The BotNet, which is used to using for sending spam mails these days, has a problem that it is not able to solve with the distributed addresses of sent mails in spam e-mails. In general, as these spam e-mails are sent from the infected Zombi PC of individual user, the sent address itself is not efficient and is meaningless to use in RBL. As an effective way to filter spam e-mail sent by BotNet, this paper analyzes URLs that contained in the original spam e-mail and proposes how to effectively improve filter rate, based on the distribution data of URL site tempting users. This paper proposes the sending mechanism of spam e-mails from BotNet and the methods to realize those types of spam e-mails. In order to gather analyzable spam e-mails, this paper also carries out an experiment by configuring trap system of spam e-mail. By analyzing spam e-mails, which have been received during the certain period of experiment, this paper shows that the expanded RBL method, using URLs that contained in spam e-mails, is effective way to improve the filter distribution of spam e-mail.
In this study, I examine overall conditions and problems of personal asset management processes by the old age people in Korea from the global perspectives. Major recommended policy implications for those are as follows.. First, the IRR (income replacement ratio) of public pensions in Korea is found to rank nearly the lowest among the OECD member countries. The relatively low fund performance compared to that of developed countries as well as this low IRR can be pointed out as major problems of public pension in Korea. It is recommended to reinforce specialty in fund management as a top priority to solve out these problems related with public pensions in Korea. Second, it is needed to set retirement pensions to be mandatory for almost all the firms in Korea to substitute for the above lower IRR of public pensions and to recover from the highest elderly poverty ratio among the OECD countries. Third, it is required to discuss about the expansion of tax refund policy application in the individual pension sector and many financial investment products under the correction of current budget control to motivate voluntary subscription for individual pension planning and to stabilize elderly lives of ordinary people in Korea. Fourth, it is required to induce market mechanism in controling price and longevity risk of reverse mortgages for the long-run sustainability.
The purpose of this study was to analyze the influence of participative leadership on the job commitment of employees and to investigate the mediating effect of psychological empowerment and the moderating effect of issue leadership. A survey was conducted on employees working in various industries and analyzed using PROCESS macro (Hayes, 2013). The reliability and validity of measurement tools were reviewed through a preliminary survey, and the timing of measurement of independent variable and dependent variable was separated to overcome the common method bias. The results of the study are as follows. First, it has been confirmed that participative leadership has a positive influence on employee's job commitment. Second, participative leadership strengthened the psychological empowerment of employees, and through it, the indirect effect of improving the employee's job commitment was confirmed. Third, the moderating effect of issue leadership was confirmed in the relationship between participative leadership and psychological empowerment. Fourth, the moderated mediating effect was confirmed that the effect of participative leadership on job commitment through psychological empowerment is reinforced by issue leadership. This study contributes to the expansion of existing leadership research by empirically identifying the mechanism between participative leadership and employee's job commitment. Implications regarding the results of the study, limitations of the study, and future study tasks were discussed.
KSII Transactions on Internet and Information Systems (TIIS)
/
v.15
no.5
/
pp.1666-1689
/
2021
The red line of Permanent Basic Farmland is the most important part in the "three-line" demarcation of China's national territorial development plan. The scientific and reasonable delineation of the red line is a major strategic measure being taken by China to improve its ability to safeguard the practical interests of farmers and guarantee national food security. The delineation of Permanent Basic Farmland zoning (DPBFZ) is essentially a multi-objective optimization problem. However, the traditional method of demarcation does not take into account the synergistic development goals of conservation of cultivated land utilization, ecological conservation, or urban expansion. Therefore, this research introduces the idea of artificial immune optimization and proposes a multi-objective model of DPBFZ red line delineation based on a clone selection algorithm. This research proposes an objective functional system consisting of these three sub-objectives: optimal quality of cropland, spatially concentrated distribution, and stability of cropland. It also takes into consideration constraints such as the red line of ecological protection, topography, and space for major development projects. The mathematical formal expressions for the objectives and constraints are given in the paper, and a multi-objective optimal decision model with multiple constraints for the DPBFZ problem is constructed based on the clone selection algorithm. An antibody coding scheme was designed according to the spatial pattern of DPBFZ zoning. In addition, the antibody-antigen affinity function, the clone mechanism, and mutation strategy were constructed and improved to solve the DPBFZ problem with a spatial optimization feature. Finally, Tongxu County in Henan province was selected as the study area, and a controlled experiment was set up according to different target preferences. The results show that the model proposed in this paper is operational in the work of delineating DPBFZ. It not only avoids the adverse effects of subjective factors in the delineation process but also provides multiple scenarios DPBFZ layouts for decision makers by adjusting the weighting of the objective function.
We proposed a numerical method for the thermo-mechanical behavior of rock fracture using a grain-based distinct element model (GBDEM) and simulated thermally induced fracture slip. The present study is the benchmark simulation performed as part of DECOVALEX-2023 Task G, which aims to develop a numerical method to estimate the coupled thermo-hydro-mechanical processes within the crystalline rock fracture network. We represented the rock sample as an assembly of Voronoi grains and calculated the interaction of the grains (blocks) and their interfaces (contacts) using a distinct element code, 3DEC. Based on an equivalent continuum approach, the micro-parameters of grains and contacts were determined to reproduce rock as an elastic material. Then, the behavior of the fracture embedded in the rock was characterized by the contacts with Coulomb shear strength and tensile strength. In the benchmark simulation, we quantitatively examined the effects of the boundary stress and thermal stress due to heat conduction on fracture behavior, focusing on the mechanism of thermally induced fracture slip. The simulation results showed that the developed numerical model reasonably reproduced the thermal expansion and thermal stress increment, the fracture stress and displacement and the effect of boundary condition. We expect the numerical model to be enhanced by continuing collaboration and interaction with other research teams of DECOVALEX-2023 Task G and validated in further study experiments.
The purpose of this study is to analyze the influence of coaching leadership on the employability of organizational members and to investigate the mediating effect of protean career attitude and the moderating effect of leader trust. A questionnaire was conducted on domestic office workers, and analysis was conducted using AMOS and Process macro (Hayes, 2013) based on the effective questionnaire of 389 people. As a result of the study, first, the direct effect between coaching leadership and employee employability was significant. Second, it was confirmed that the mediating effect of coaching leadership strengthening the protean career attitude of members and improving employability through this was significant. Third, the moderating effect of leader trust was confirmed in the relationship between coaching leadership and protean career attitude. Fourth, the moderated mediating effect of coaching leadership on employability through protean career attitude was reinforced by leader trust was confirmed. This study contributes to the expansion of leadership research by empirically identifying the mechanism between coaching leadership and employability of organizational members, which has been lacking in research. In addition, theoretical and practical implications of the research results, limitations of the research, and directions for future research were presented.
Kim, Junmo;Kim, Boyeon;Jung, Cheong-Ha;Kim, Gu-sung;Kim, Taek-Soo
Journal of the Microelectronics and Packaging Society
/
v.29
no.3
/
pp.25-29
/
2022
Recently, the importance of electronic packaging technology has been attracting attention, and heterogeneous integration technology in which chips are stacked out-of-plane direction is being applied to the electronic packaging field. The 2.5D integration circuit is a technology for stacking chips using an interposer including TSV, and is widely used already. Therefore, it is necessary to make the interposer mechanically reliable in the packaging process that undergoes various thermal processes and mechanical loadings. Considering the structural characteristics of the interposer on which several thin films are deposited, thermal stress due to the difference in thermal expansion coefficients of materials can have a great effect on reliability. In this study, the mechanical reliability of the metal pad for wire bonding on the silicon interposer against thermal stress was evaluated. After heating the interposer to the solder reflow temperature, the delamination of the metal pad that occurred during cooling was observed and the mechanism was investigated. In addition, it was confirmed that the high cooling rate and the defect caused by handling promote delamination of the metal pads.
The cryosuction (negative pore pressure) in freezing soils causes groundwater migration from the frozen fringe to freezing front for ice lens formation. Frost heave and heaving pressure by ice lens cause damage to ground infrastructure. In order to prevent damage by the frost heave, various frost susceptibility criteria have been proposed. The SP (Segregation Potential) is the most widely used classification criterion for frost susceptibility in cold regions. The expansion of the ice lens by the migration of the groundwater is a key role in frost heave mechanism, and thus it is necessary to evaluate the hydraulic conductivity. In this paper, soil mixtures of coarse-fines (sand-silt) were prepared in various weight fractions and used for frost heave and column permeability test. For each case, the SP and the hydraulic conductivity were derived and correlations were analyzed. As a results, the transition threshold of the SP and the hydraulic conductivity were shown at 20% and 50% of the silt weight fraction, respectively. Although there are difference between these transition thresholds, these two coefficients show a specific correlation. In the future, additional study should be conducted for detailed analysis of the threshold transition values between SP and hydraulic conductivity.
This study aims to understand a gamer participatory mechanism by comparing Gamer Participation 1.0(Bartz Liberation War) and Gamer Participation 2.0(Truck Demonstration). The process of gamers' political participation is analyzed by applying the communication-mediated O-S-O-R model with the case of gamers' truck protests caused by probability items' issue. This study found out that changes in the social, technological, and discursive layers that constitute the gamer's communication ecology led to diversification of communication channels and changes in the perception of games. Gamers utilized the technological layer of the media environment that presupposes immediate mutual communication, expressed opinions on issues in the mobilization process, and shared the necessity of participation to derive collective mobilization. Through communication, gamers were able to participate in socio-political issues with high participation thresholds. This study is significant in that it discussed the gamers' democratic citizenship and role as issue publics. The study suggests that the need for theoretical and methodological expansion to analyze various participatory cases.
본 웹사이트에 게시된 이메일 주소가 전자우편 수집 프로그램이나
그 밖의 기술적 장치를 이용하여 무단으로 수집되는 것을 거부하며,
이를 위반시 정보통신망법에 의해 형사 처벌됨을 유념하시기 바랍니다.
[게시일 2004년 10월 1일]
이용약관
제 1 장 총칙
제 1 조 (목적)
이 이용약관은 KoreaScience 홈페이지(이하 “당 사이트”)에서 제공하는 인터넷 서비스(이하 '서비스')의 가입조건 및 이용에 관한 제반 사항과 기타 필요한 사항을 구체적으로 규정함을 목적으로 합니다.
제 2 조 (용어의 정의)
① "이용자"라 함은 당 사이트에 접속하여 이 약관에 따라 당 사이트가 제공하는 서비스를 받는 회원 및 비회원을
말합니다.
② "회원"이라 함은 서비스를 이용하기 위하여 당 사이트에 개인정보를 제공하여 아이디(ID)와 비밀번호를 부여
받은 자를 말합니다.
③ "회원 아이디(ID)"라 함은 회원의 식별 및 서비스 이용을 위하여 자신이 선정한 문자 및 숫자의 조합을
말합니다.
④ "비밀번호(패스워드)"라 함은 회원이 자신의 비밀보호를 위하여 선정한 문자 및 숫자의 조합을 말합니다.
제 3 조 (이용약관의 효력 및 변경)
① 이 약관은 당 사이트에 게시하거나 기타의 방법으로 회원에게 공지함으로써 효력이 발생합니다.
② 당 사이트는 이 약관을 개정할 경우에 적용일자 및 개정사유를 명시하여 현행 약관과 함께 당 사이트의
초기화면에 그 적용일자 7일 이전부터 적용일자 전일까지 공지합니다. 다만, 회원에게 불리하게 약관내용을
변경하는 경우에는 최소한 30일 이상의 사전 유예기간을 두고 공지합니다. 이 경우 당 사이트는 개정 전
내용과 개정 후 내용을 명확하게 비교하여 이용자가 알기 쉽도록 표시합니다.
제 4 조(약관 외 준칙)
① 이 약관은 당 사이트가 제공하는 서비스에 관한 이용안내와 함께 적용됩니다.
② 이 약관에 명시되지 아니한 사항은 관계법령의 규정이 적용됩니다.
제 2 장 이용계약의 체결
제 5 조 (이용계약의 성립 등)
① 이용계약은 이용고객이 당 사이트가 정한 약관에 「동의합니다」를 선택하고, 당 사이트가 정한
온라인신청양식을 작성하여 서비스 이용을 신청한 후, 당 사이트가 이를 승낙함으로써 성립합니다.
② 제1항의 승낙은 당 사이트가 제공하는 과학기술정보검색, 맞춤정보, 서지정보 등 다른 서비스의 이용승낙을
포함합니다.
제 6 조 (회원가입)
서비스를 이용하고자 하는 고객은 당 사이트에서 정한 회원가입양식에 개인정보를 기재하여 가입을 하여야 합니다.
제 7 조 (개인정보의 보호 및 사용)
당 사이트는 관계법령이 정하는 바에 따라 회원 등록정보를 포함한 회원의 개인정보를 보호하기 위해 노력합니다. 회원 개인정보의 보호 및 사용에 대해서는 관련법령 및 당 사이트의 개인정보 보호정책이 적용됩니다.
제 8 조 (이용 신청의 승낙과 제한)
① 당 사이트는 제6조의 규정에 의한 이용신청고객에 대하여 서비스 이용을 승낙합니다.
② 당 사이트는 아래사항에 해당하는 경우에 대해서 승낙하지 아니 합니다.
- 이용계약 신청서의 내용을 허위로 기재한 경우
- 기타 규정한 제반사항을 위반하며 신청하는 경우
제 9 조 (회원 ID 부여 및 변경 등)
① 당 사이트는 이용고객에 대하여 약관에 정하는 바에 따라 자신이 선정한 회원 ID를 부여합니다.
② 회원 ID는 원칙적으로 변경이 불가하며 부득이한 사유로 인하여 변경 하고자 하는 경우에는 해당 ID를
해지하고 재가입해야 합니다.
③ 기타 회원 개인정보 관리 및 변경 등에 관한 사항은 서비스별 안내에 정하는 바에 의합니다.
제 3 장 계약 당사자의 의무
제 10 조 (KISTI의 의무)
① 당 사이트는 이용고객이 희망한 서비스 제공 개시일에 특별한 사정이 없는 한 서비스를 이용할 수 있도록
하여야 합니다.
② 당 사이트는 개인정보 보호를 위해 보안시스템을 구축하며 개인정보 보호정책을 공시하고 준수합니다.
③ 당 사이트는 회원으로부터 제기되는 의견이나 불만이 정당하다고 객관적으로 인정될 경우에는 적절한 절차를
거쳐 즉시 처리하여야 합니다. 다만, 즉시 처리가 곤란한 경우는 회원에게 그 사유와 처리일정을 통보하여야
합니다.
제 11 조 (회원의 의무)
① 이용자는 회원가입 신청 또는 회원정보 변경 시 실명으로 모든 사항을 사실에 근거하여 작성하여야 하며,
허위 또는 타인의 정보를 등록할 경우 일체의 권리를 주장할 수 없습니다.
② 당 사이트가 관계법령 및 개인정보 보호정책에 의거하여 그 책임을 지는 경우를 제외하고 회원에게 부여된
ID의 비밀번호 관리소홀, 부정사용에 의하여 발생하는 모든 결과에 대한 책임은 회원에게 있습니다.
③ 회원은 당 사이트 및 제 3자의 지적 재산권을 침해해서는 안 됩니다.
제 4 장 서비스의 이용
제 12 조 (서비스 이용 시간)
① 서비스 이용은 당 사이트의 업무상 또는 기술상 특별한 지장이 없는 한 연중무휴, 1일 24시간 운영을
원칙으로 합니다. 단, 당 사이트는 시스템 정기점검, 증설 및 교체를 위해 당 사이트가 정한 날이나 시간에
서비스를 일시 중단할 수 있으며, 예정되어 있는 작업으로 인한 서비스 일시중단은 당 사이트 홈페이지를
통해 사전에 공지합니다.
② 당 사이트는 서비스를 특정범위로 분할하여 각 범위별로 이용가능시간을 별도로 지정할 수 있습니다. 다만
이 경우 그 내용을 공지합니다.
제 13 조 (홈페이지 저작권)
① NDSL에서 제공하는 모든 저작물의 저작권은 원저작자에게 있으며, KISTI는 복제/배포/전송권을 확보하고
있습니다.
② NDSL에서 제공하는 콘텐츠를 상업적 및 기타 영리목적으로 복제/배포/전송할 경우 사전에 KISTI의 허락을
받아야 합니다.
③ NDSL에서 제공하는 콘텐츠를 보도, 비평, 교육, 연구 등을 위하여 정당한 범위 안에서 공정한 관행에
합치되게 인용할 수 있습니다.
④ NDSL에서 제공하는 콘텐츠를 무단 복제, 전송, 배포 기타 저작권법에 위반되는 방법으로 이용할 경우
저작권법 제136조에 따라 5년 이하의 징역 또는 5천만 원 이하의 벌금에 처해질 수 있습니다.
제 14 조 (유료서비스)
① 당 사이트 및 협력기관이 정한 유료서비스(원문복사 등)는 별도로 정해진 바에 따르며, 변경사항은 시행 전에
당 사이트 홈페이지를 통하여 회원에게 공지합니다.
② 유료서비스를 이용하려는 회원은 정해진 요금체계에 따라 요금을 납부해야 합니다.
제 5 장 계약 해지 및 이용 제한
제 15 조 (계약 해지)
회원이 이용계약을 해지하고자 하는 때에는 [가입해지] 메뉴를 이용해 직접 해지해야 합니다.
제 16 조 (서비스 이용제한)
① 당 사이트는 회원이 서비스 이용내용에 있어서 본 약관 제 11조 내용을 위반하거나, 다음 각 호에 해당하는
경우 서비스 이용을 제한할 수 있습니다.
- 2년 이상 서비스를 이용한 적이 없는 경우
- 기타 정상적인 서비스 운영에 방해가 될 경우
② 상기 이용제한 규정에 따라 서비스를 이용하는 회원에게 서비스 이용에 대하여 별도 공지 없이 서비스 이용의
일시정지, 이용계약 해지 할 수 있습니다.
제 17 조 (전자우편주소 수집 금지)
회원은 전자우편주소 추출기 등을 이용하여 전자우편주소를 수집 또는 제3자에게 제공할 수 없습니다.
제 6 장 손해배상 및 기타사항
제 18 조 (손해배상)
당 사이트는 무료로 제공되는 서비스와 관련하여 회원에게 어떠한 손해가 발생하더라도 당 사이트가 고의 또는 과실로 인한 손해발생을 제외하고는 이에 대하여 책임을 부담하지 아니합니다.
제 19 조 (관할 법원)
서비스 이용으로 발생한 분쟁에 대해 소송이 제기되는 경우 민사 소송법상의 관할 법원에 제기합니다.
[부 칙]
1. (시행일) 이 약관은 2016년 9월 5일부터 적용되며, 종전 약관은 본 약관으로 대체되며, 개정된 약관의 적용일 이전 가입자도 개정된 약관의 적용을 받습니다.