• Title/Summary/Keyword: SCA type 2

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Isolation and Characterization of a Calmodulin-binding Ca2+-ATPase 2 (SCA2) in Soybean (칼모듈린에 결합하는 대두 Ca2+-ATPase 2 (SCA2)의 분리 및 특성 분석)

  • Park, Hyeong-Cheol;Kim, Ho-Soo;Lee, Sang-Min;Cho, Hyeon-Seol;Chung, Woo-Sik
    • Journal of Life Science
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    • v.21 no.5
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    • pp.671-677
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    • 2011
  • We previously reported the isolation and characterization of a gene, SCA1 (for soybean $Ca^{2+}$-ATPase 1), encoding a calmodulin-regulated $Ca^{2+}$-ATPase that is located in the plasma membrane in soybean. Here, a $Ca^{2+}$-ATPase designated as SCA2 was isolated from soybean. The two $Ca^{2+}$-ATPases, SCA1 and SCA2, share a remarkably high degree of similarity (78%). Ten transmemebrane domains were predicted by hydropathy analysis. Using gel overlay assays, CaM was found to bind to SCA2 in a $Ca^{2+}$-dependent manner. Southern blot analysis revealed the presence of two copies of the $Ca^{2+}$-ATPase gene in the soybean genome. An N-terminal truncation mutant that deletes sequence through the putative calmodulin binding site was able to complement a yeast mutant (K616) that was deficient in two endogenous $Ca^{2+}$ pumps. Our results indicate that SCA2 is structurally highly conserved with type IIB $Ca^{2+}$ pumps in plants.

A Case Report on Spinocerebellar Ataxia Type 2 (제2형 척수소뇌실조 환자 치험 1례)

  • An, Da-young;Han, Seung-hee;Nam, Hyun-seo;Han, Su-zy;Kim, Mi-kyung;Sun, Seung-ho
    • The Journal of Internal Korean Medicine
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    • v.42 no.5
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    • pp.784-792
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    • 2021
  • Objectives: The purpose of this study was to investigate a case of a patient with spinocerebellar ataxia (SCA) type 2 whose condition improved following treatment with Korean medicine. Methods: A 25-year-old man, diagnosed with SCA type 2, was treated with herbal medicine (Yukmijiwhang-tang-gami), acupuncture, and physical treatment. The therapeutic effect was evaluated using the Berg Balance Scale (BBS) and gait status. Results: Following treatment, the BBS score increased, and gait ataxia improved. Conclusions: This case study suggests that Korean medicine could be effective for relieving symptoms of SCA type 2.

A Case of Spinocerebellar Ataxia Type 2 with Slowed Saccades (느린 홱보기를 동반한 척수소뇌실조 2형 1예)

  • Nam, Jungmoo;Kim, Byung-Kun;Koo, Ja-Seong;Park, Jongmoo;Lee, JungJu;Kwon, Ohyun
    • Annals of Clinical Neurophysiology
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    • v.9 no.2
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    • pp.102-104
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    • 2007
  • Spinocerebellar ataxia type 2 (SCA2) is characterized by progressive cerebellar ataxia and slow saccades. A 40-year-old woman presented with progressive gait disturbance and ataxia over 15 years. Neurologic examination revealed scanning speech, ataxia, and hyporeflexia. Brain CT showed diffuse atrophy of the cerebellum. Electronystagmography demonstrated slowed saccades with normal accuracy and delayed latency. The diagnosis of SCA2 was confirmed by the genetic test. Documentation of slow saccades may help differentiation among SCA subgroups.

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Spinocerebellar ataxia 7 (SCA7) (척수소뇌성 운동실조증 제7형)

  • Seon-Yong, Jeong;Seok-Hun, Jang;Hyon-J., Kim
    • Journal of Genetic Medicine
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    • v.4 no.1
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    • pp.22-37
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    • 2007
  • 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.

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Proteasome Function Is Inhibited by Polyglutamine-expanded Ataxin-1, the SCA1 Gene Product

  • Park, Yongjae;Hong, Sunghoi;Kim, Sung-Jo;Kang, Seongman
    • Molecules and Cells
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    • v.19 no.1
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    • pp.23-30
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    • 2005
  • Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder caused by expansion of the polyglutamine tract in the SCA1 gene product, ataxin-1. Using d2EGFP, a short-lived enhanced green fluorescent protein, we investigated whether polyglutamine-expanded ataxin-1 affects the function of the proteasome, a cellular multicatalytic protease that degrades most misfolded proteins and regulatory proteins. In Western blot analysis and immunofluorescence experiments, d2EGFP was less degraded in HEK 293T cells transfected with ataxin-1(82Q) than in cells transfected with lacZ or empty vector controls. To test whether the stability of the d2EGFP protein was due to aggregation of ataxin-1, we constructed a plasmid carrying $ataxin-1-{\Delta}114$, lacking the self-association region (SAR), and examined degradation of the d2EGFP. Both the level of $ataxin-1-{\Delta}114$ aggregates and the amount of d2EGFP were drastically reduced in cells containing $ataxin-1-{\Delta}114$. Furthermore, d2EGFP localization experiments showed that polyglutamine-expanded ataxin-1 inhibited the general function of the proteasome activity. Taken together, these results demonstrate that polyglutamine-expanded ataxin-1 decreases the activity of the proteasome, implying that a disturbance in the ubiquitin-proteasome pathway is directly involved in the development of spinocerebellar ataxia type1.

Case Report of a Type 3 Spinocerebellar Ataxia Patient Treated with Traditional Korean Medicine (제 3형 척수소뇌실조 환자의 한방 치험 1례)

  • Hur, Hee-soo;Lee, Myeong-hwa;Kim, Kyoung-min;Kim, Young-kyun
    • The Journal of Internal Korean Medicine
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    • v.37 no.2
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    • pp.322-329
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    • 2016
  • Objective: This study reports on one clinical case of type 3 spinocerebellar ataxia (SCA) to examine the effectiveness of herbal medicine and traditional Korean treatments.Methods: A patient with type 3 spinocerebellar ataxia was treated using traditional Korean medicine options such as acupuncture and Hyangsayukgunja-tang. The effects on type 3 spinocerebellar ataxia were measured using the numeric rating scale (NRS) and ambulatory status.Results: Improvements in NRS and ambulatory status were observed after treatment.Conclusion: According to this study, traditional Korean medicine may be effective in the treatment of type 3 spinocerebellar ataxia.

Genetic Variance and Combining Ability of Shattering and Growth Characters by Diallel Crosses of Sesame (이면교잡에 의한 참깨의 탈립성 및 생육형질의 유전변이와 조합능력)

  • Kim, Dong-Hwi;Kang, Chul-Whan;Shim, Kang-Bo;Park, Chang-Hwan;Lee, Sung-Woo;Seong, Nak-Sul
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.51 no.7
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    • pp.652-657
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    • 2006
  • Combining ability and heterosis for $F_{1}$ population obtained $5{\times}5$ half diallel cross in sesame were estimated for six quantitative characters. The parental varieties used for diallel cross were Yangbaek and Ansan of normal type, Suwon 177 and 195 of placenta adhesion type (PA) and SIG960320-5-1-1 of indehiscent type (ID). PA and ID type are shattering-resistant sesames and Yangbaek and Ansan are shattering sesames. The high heterosis of $F_{1}$ hybrids was expressed in the number of capsules per plant (NC), grain yield per plant (GY) and number of branches per plant (NB). Shattering.ate (SR) showed high positive heterosis toward increase of seeds shattering in all the combination of $F_{1}$ generation which was presumed because the characters connected with shattering resistance of seeds were governed by recessive gene. At the analysis of combining ability, highly significant general combining ability (GCA) effects were observed on all the characters in $F_{1}$ generation, significant specific combining ability (SCA) effects were observed on the NC, GY and SR. GCA variances were greater than SCA variance, which suggested that additive gene effects were greater than other nonadditive gene effects. Considering the estimated GCA effects, Suwon 195 (PA type) was found to be good parent for SR and GY, SIG960320-5-1-1 (ID type) was poor for GY and plant height. SCA effect for NC was great in Yangbaek/Suwon 195, SCA for GY was great in Ansan/SIG960320-5-1-1, and SCA for decrease of SR was great in Ansan/Suwon177.

Comparative Analysis of ViSCa Platform-based Mobile Payment Service with other Cases (스마트카드 가상화(ViSCa) 플랫폼 기반 모바일 결제 서비스 제안 및 타 사례와의 비교분석)

  • Lee, June-Yeop;Lee, Kyoung-Jun
    • Journal of Intelligence and Information Systems
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    • v.20 no.2
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    • pp.163-178
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    • 2014
  • Following research proposes "Virtualization of Smart Cards (ViSCa)" which is a security system that aims to provide a multi-device platform for the deployment of services that require a strong security protocol, both for the access & authentication and execution of its applications and focuses on analyzing Virtualization of Smart Cards (ViSCa) platform-based mobile payment service by comparing with other similar cases. At the present day, the appearance of new ICT, the diffusion of new user devices (such as smartphones, tablet PC, and so on) and the growth of internet penetration rate are creating many world-shaking services yet in the most of these applications' private information has to be shared, which means that security breaches and illegal access to that information are real threats that have to be solved. Also mobile payment service is, one of the innovative services, has same issues which are real threats for users because mobile payment service sometimes requires user identification, an authentication procedure and confidential data sharing. Thus, an extra layer of security is needed in their communication and execution protocols. The Virtualization of Smart Cards (ViSCa), concept is a holistic approach and centralized management for a security system that pursues to provide a ubiquitous multi-device platform for the arrangement of mobile payment services that demand a powerful security protocol, both for the access & authentication and execution of its applications. In this sense, Virtualization of Smart Cards (ViSCa) offers full interoperability and full access from any user device without any loss of security. The concept prevents possible attacks by third parties, guaranteeing the confidentiality of personal data, bank accounts or private financial information. The Virtualization of Smart Cards (ViSCa) concept is split in two different phases: the execution of the user authentication protocol on the user device and the cloud architecture that executes the secure application. Thus, the secure service access is guaranteed at anytime, anywhere and through any device supporting previously required security mechanisms. The security level is improved by using virtualization technology in the cloud. This virtualization technology is used terminal virtualization to virtualize smart card hardware and thrive to manage virtualized smart cards as a whole, through mobile cloud technology in Virtualization of Smart Cards (ViSCa) platform-based mobile payment service. This entire process is referred to as Smart Card as a Service (SCaaS). Virtualization of Smart Cards (ViSCa) platform-based mobile payment service virtualizes smart card, which is used as payment mean, and loads it in to the mobile cloud. Authentication takes place through application and helps log on to mobile cloud and chooses one of virtualized smart card as a payment method. To decide the scope of the research, which is comparing Virtualization of Smart Cards (ViSCa) platform-based mobile payment service with other similar cases, we categorized the prior researches' mobile payment service groups into distinct feature and service type. Both groups store credit card's data in the mobile device and settle the payment process at the offline market. By the location where the electronic financial transaction information (data) is stored, the groups can be categorized into two main service types. First is "App Method" which loads the data in the server connected to the application. Second "Mobile Card Method" stores its data in the Integrated Circuit (IC) chip, which holds financial transaction data, which is inbuilt in the mobile device secure element (SE). Through prior researches on accept factors of mobile payment service and its market environment, we came up with six key factors of comparative analysis which are economic, generality, security, convenience(ease of use), applicability and efficiency. Within the chosen group, we compared and analyzed the selected cases and Virtualization of Smart Cards (ViSCa) platform-based mobile payment service.

Intranasal Immunization With Nanoparticles Containing an Orientia tsutsugamushi Protein Vaccine Candidate and a Polysorbitol Transporter Adjuvant Enhances Both Humoral and Cellular Immune Responses

  • Cheol Gyun Kim;Won Kyong Kim;Narae Kim;Young Jin Pyung;Da-Jeong Park;Jeong-Cheol Lee;Chong-Su Cho;Hyuk Chu;Cheol-Heui Yun
    • IMMUNE NETWORK
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    • v.23 no.6
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    • pp.47.1-47.16
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    • 2023
  • Scrub typhus, a mite-borne infectious disease, is caused by Orientia tsutsugamushi. Despite many attempts to develop a protective strategy, an effective preventive vaccine has not been developed. The identification of appropriate Ags that cover diverse antigenic strains and provide long-lasting immunity is a fundamental challenge in the development of a scrub typhus vaccine. We investigated whether this limitation could be overcome by harnessing the nanoparticle-forming polysorbitol transporter (PST) for an O. tsutsugamushi vaccine strategy. Two target proteins, 56-kDa type-specific Ag (TSA56) and surface cell Ag A (ScaA) were used as vaccine candidates. PST formed stable nano-size complexes with TSA56 (TSA56-PST) and ScaA (ScaA-PST); neither exhibited cytotoxicity. The formation of Ag-specific IgG2a, IgG2b, and IgA in mice was enhanced by intranasal vaccination with TSA56-PST or ScaA-PST. The vaccines containing PST induced Ag-specific proliferation of CD8+ and CD4+ T cells. Furthermore, the vaccines containing PST improved the mouse survival against O. tsutsugamushi infection. Collectively, the present study indicated that PST could enhance both Ag-specific humoral immunity and T cell response, which are essential to effectively confer protective immunity against O. tsutsugamushi infection. These findings suggest that PST has potential for use in an intranasal vaccination strategy.

A Two-Step Call Admission Control Scheme using Priority Queue in Cellular Networks (셀룰러 이동망에서의 우선순위 큐 기반의 2단계 호 수락 제어 기법)

  • 김명일;김성조
    • Journal of KIISE:Information Networking
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    • v.30 no.4
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    • pp.461-473
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    • 2003
  • Multimedia applications are much more sensitive to QoS(Quality of Service) than text based ones due to their data continuity. In order to provide a fast moving MH(Mobil Host) using multimedia application with a consistent QoS,an efficient call admission mechanism is in need. This paper proposes the 2SCA(2-Step Call Admission) scheme based on cal admission scheme using pripority to guarantee the consistent QoS for mobile multimedia applications. A calls of MH are classified new calls, hand-off calls, and QoS upgrading calls. The 2SCA is composed of the basic call admission and advanced call admission; the former determines the call admission based on bandwidth available in each cell and the latter determines the call admission by applying DTT(Delay Tolerance Time), PQeueu(Priority Queue), and UpQueue(Upgrade Queue) algorithm according to the type of each call blocked at the basic call admission stage. In order to evaluate the performance of our mechanism, we measure the metrics such as the dropping probability of new calls, dropping probability of hand-off calls, and bandwidth utilization. The result shows that the performance of our mechanism is superior to that of existing mechanisms such as CSP(Complete Sharing Policy), GCP(Guard Channel Policy) and AGCP(Adaptive Guard Channel Policy).