• 제목/요약/키워드: Complement 3

검색결과 944건 처리시간 0.032초

Susceptibility of KSHV-Infected PEL Cell Lines to the Human Complement System

  • Yoo, Seung-Min;Jeon, Hyungtaek;Lee, Suhyuk;Lee, Myung-Shin
    • Journal of Microbiology and Biotechnology
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    • 제26권3호
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    • pp.618-626
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    • 2016
  • Pleural effusion lymphoma (PEL) is a rare B-cell lymphoma that has a very poor prognosis with a median survival time of around 6 months. PEL is caused by Kaposi's sarcoma-associated herpesvirus, and is often co-infected with the Epstein Barr virus. The complement system is fundamental in the innate immune system against pathogen invasion and tumor development. In the present study, we investigated the activation of the complement system in PEL cells using human serum complements. Interestingly, two widely used PEL cell lines, BCP-1 and BCBL-1, showed different susceptibility to the complement system, which may be due to CD46 expression on their cell membranes. Complement activation did not induce apoptosis but supported cell survival considerably. Our results demonstrated the susceptibility of PEL to the complement system and its underlying mechanisms, which would provide insight into understanding the pathogenesis of PEL.

Atypical Hemolytic Uremic Syndrome in a 13-year-old Lao Girl: A Case Report

  • Kedsatha, Philavanh;Cheong, Hae Il;Choi, Yong
    • Childhood Kidney Diseases
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    • 제23권1호
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    • pp.43-47
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    • 2019
  • Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

Effectiveness of Cyclosporine in a 10-year-old Girl with C3 Glomerulopathy

  • Jang, Kyung Mi;Park, Yong Hoon
    • Childhood Kidney Diseases
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    • 제21권2호
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    • pp.160-164
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    • 2017
  • C3 glomerulopathy (C3G) is a recently defined pathological entity characterized by C3 accumulation with absent or scant immunoglobulin deposition, leading to variable glomerular inflammation. The clinical presentation of patients with C3G is highly variable, as they may present with symptoms ranging from microscopic or mild proteinuria to full-blown nephrotic syndrome, with or without renal impairment. However, there is no consensus recommendation for specific treatment in children with C3G. Recently, new therapies have been suggested to target complement pathways, owing to an improvement in the understanding of the pathogenesis of C3G. C3G complement blockade with eculizumab, a monoclonal antibody targeted against complement C5, inhibits activation of the alternative complement pathway. We could not use eculizumab owing to its high price; thus, we administered oral prednisolone and mycophenolate mofetil (MMF). MMF was replaced with cyclosporine because proteinuria persisted, with a consistently low serum C3 level; we tapered off the prednisolone because of a Cushingoid appearance and amenorrhea. Thereafter, proteinuria improved, and the serum C3 level returned to normal. Thus, we report the effectiveness of cyclosporine in a patient with C3G and an inadequate response to prednisolone and MMF, who was detected via school urinary screening.

Moieties of Complement iC3b Recognized by the I-domain of Integrin αXβ2

  • Choi, Jeongsuk;Buyannemekh, Dolgorsuren;Nham, Sang-Uk
    • Molecules and Cells
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    • 제43권12호
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    • pp.1023-1034
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    • 2020
  • Complement fragment iC3b serves as a major opsonin for facilitating phagocytosis via its interaction with complement receptors CR3 and CR4, also known by their leukocyte integrin family names, αMβ2 and αXβ2, respectively. Although there is general agreement that iC3b binds to the αM and αX I-domains of the respective β2-integrins, much less is known regarding the regions of iC3b contributing to the αX I-domain binding. In this study, using recombinant αX I-domain, as well as recombinant fragments of iC3b as candidate binding partners, we have identified two distinct binding moieties of iC3b for the αX I-domain. They are the C3 convertase-generated N-terminal segment of the C3b α'-chain (α'NT) and the factor I cleavage-generated N-terminal segment in the CUBf region of α-chain. Additionally, we have found that the CUBf segment is a novel binding moiety of iC3b for the αM I-domain. The CUBf segment shows about a 2-fold higher binding activity than the α'NT for αX I-domain. We also have shown the involvement of crucial acidic residues on the iC3b side of the interface and basic residues on the I-domain side.

C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report

  • Hyun, HyeSun;Kang, Hee Gyung;Cho, UiJu;Ha, Il-Soo;Cheong, Hae Il
    • Childhood Kidney Diseases
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    • 제25권1호
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    • pp.29-34
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    • 2021
  • C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

Survival of Miamiensis avidus (Ciliophora: Scuticociliatia) from antibody-dependent complement killing

  • Lee, Eun Hye;Kang, Yue Jai;Kim, Ki Hong
    • 한국어병학회지
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    • 제28권3호
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    • pp.171-174
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    • 2015
  • Previously, we had reported that some Miamiensis avidus, a major pathogen of scuticociliatosis in cultured olive flounder, strongly agglutinated by flounder immune sera could escape from the agglutinated mass within a few hours. In the present study, we observed that M. avidus not only escaped from the agglutinated mass but also conducted division(s) before shedding its old covering. Furthermore, ciliates that survived the antibody-dependent complement killing (ADCK) assay were not killed even when re-exposed to a freshly prepared ADCK assay. This result suggests that the liberated ciliates from the ADCK assay might change not only their i-antigen types but also the epitopes of major surface antigens, which debilitate antibody-mediated complement killing ability.

A complement to Hoek-Brown failure criterion for strength prediction in anisotropic rock

  • Bagheripour, Mohammad Hossein;Rahgozar, Reza;Pashnesaz, Hassan;Malekinejad, Mohsen
    • Geomechanics and Engineering
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    • 제3권1호
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    • pp.61-81
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    • 2011
  • In this paper, a complement to the Hoek-Brown criterion is proposed in order to derive the strength of anisotropic rock from strength of the corresponding truly intact rock. The complement is a decay function, which unlike other modifications or suggestions made in the past, is multiplied to the function of the original Hoek-Brown failure criterion for intact rock. This results in a combined and extended form of the criterion which describes the strength of anisotropic rock as a varying fraction of the corresponding truly intact rock strength. Statistical procedures and in particular regression analyses were conducted into data obtained in experiments conducted in the current research program and those collected from the literature in order to define the Hoek-Brown's criterion complement. The complement function was best described by a simple polynomial including only three constants to be empirically evaluated. Further investigations also showed that these constants can be related to the other readily available parameters of rock material which further facilitate determining the constants. A great and prime advantage of the proposed complement is that it is mathematically simple including the least possible number of empirical constants which are easily estimated with minimum experimental effort. Moreover, proposed concept does not suggests any change to the original Hoek-Brown criterion itself or its constants and serves whenever anisotropy does exist in the rock. This further implies on the possibility of using any other failure criterion for intact rock in conjunction with the compliment to reach the strength of anisotropic rock.

High-Throughput Low-Complexity Successive-Cancellation Polar Decoder Architecture using One's Complement Scheme

  • Kim, Cheolho;Yun, Haram;Ajaz, Sabooh;Lee, Hanho
    • JSTS:Journal of Semiconductor Technology and Science
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    • 제15권3호
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    • pp.427-435
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    • 2015
  • This paper presents a high-throughput low-complexity decoder architecture and design technique to implement successive-cancellation (SC) polar decoding. A novel merged processing element with a one's complement scheme, a main frame with optimal internal word length, and optimized feedback part architecture are proposed. Generally, a polar decoder uses a two's complement scheme in merged processing elements, in which a conversion between two's complement and sign-magnitude requires an adder. However, the novel merged processing elements do not require an adder. Moreover, in order to reduce hardware complexity, optimized main frame and feedback part approaches are also presented. A (1024, 512) SC polar decoder was designed and implemented using 40-nm CMOS standard cell technology. Synthesis results show that the proposed SC polar decoder can lead to a 13% reduction in hardware complexity and a higher clock speed compared to conventional decoders.

수막구균 뇌수막염과 보체 결핍이 동반된 막증식성 사구체신염 1례 (A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis)

  • 권상미;박관규;이경훈
    • Childhood Kidney Diseases
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    • 제10권1호
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    • pp.45-51
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    • 2006
  • 저자들은 C3의 감소와 육안적 혈뇨를 보인 환아에서 현미경적 혈뇨가 지속되면서 8주 후 시행한 검사에서도 C3의 감소가 지속되어 MPGN을 의심하고 신생검 예정이었다. 추적 관찰 중 수막구균 뇌수막염이 동반되었고, C3와 C5의 지속적인 감소와 C7과 C9의 일시적인 감소를 확인하였으며, 신생검에서 1형 MPGN으로 확인되었다. 본 증례의 아쉬운 점은 nephritic factor의 존재나 factor H의 결핍을 입증하여야 하나 아직까지 국내에서는 시행되지 않아 확인은 하지 못하였다. 앞으로 국내에서도 nephritic factor나 다양한 보체계(complement profile)에 대한 검사들이 시행되어야 할 것이다. 이제까지 국내에서는 후기 보체 결핍에 의하여 수막구균 뇌수막의 발생 증례 보고는 2004년 이후 여러 차례 있었지만, MPGN 환자에서 수막구균의 감염은 국내에 보고된 바가 없다. 본 증례를 통해 저보체혈증이 지속되는 일부의 MPGN 환자에서는 수막구균 감염 위험성이 존재하며, nephritic factor 검사와 함께 후기 보체 결핍에 대한 검사가 필요하다고 생각한다. 또한 수막구균성 뇌수막염에 이환된 모든 환자에 대해서 보체 검사를 시행하고 이런 환자에서 주기적인 소변 검사를 통하여 이상이 있을시 MPGN의 가능성을 고려하여 신생검을 고려하여야 할 것이다.

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Linguistic Description and Theory

  • Nakajima, Heizo
    • 한국영어학회지:영어학
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    • 제1권3호
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    • pp.349-368
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    • 2001
  • We have brought up several distinct types of English clausal constructions, and have been lead to the descriptive generalization in (14),repeated here as (33): (33) Reduced clauses cannot occur in non-complement positions. The generalization in (33) refers to two theory-internal notions, reduced clauses and non-complement positions. Both notions are concerned with the composition of syntactic structures to be defined by X-bar theory. Without these theoretical notions, it would be difficult to describe in a general form the fact that certain types of complement clauses-namely, null-that clauses, if-clauses, Acc-ing gerund, ECM complement clauses, and Raising complement clauses-cannot occur in particular syntactic positions. Instead, one would have to describe this fact for each clause type, in such a way that null-that clauses cannot occur in such and such positions, and if-clauses cannot occur in such and such positions, and Acc-ing gerund cannot occur in such and such positions, and so on, although the positions in which they cannot occur are totally the same. Given the terminology of X-bar theory, however, it has turned out that these types of complement clauses are all reduced clauses, and the positions where they cannot occur are all non-complement positions. Then, the generalization has obtained that reduced clauses cannot occur in non-complement positions. It is a theoretical issue, and differs depending upon theories, how to explain why such a descriptive generalization holds at all. Hopefully, the demonstration here provides a piece of evidence showing that a theory or a particular theoretical nation plays an important role in the description of linguistic facts. Moreover, I have made a crucial prediction on the basis of the well-accepted theoretical assumption the ECM complement clauses and Raising complement clauses are reduced clauses; namely, the prediction that these types of clauses cannot occur in non-complement position. The prediction based upon the theoretical assumption is actually borne out, as illustrated earlier. The illustration of the prediction, I hope, shows that a theory or a particular theoretical assumption, coupled with another theoretical assumption, allows us to make some interesting predictions. Predictions serve to widen a range of linguistic facts to be described. A theory plays a crucial part in finding out interesting facts as well as in describing them in some general forms. Finally, let me state a few words as to the recent generative theory in connection with linguistic description. The recent generative theory is getting more and more abstract. I think it is moving toward a good direction as cognitive science. It will contribute, among others, to the inquiry into what is knowledge that is very specific to language faculty, and into how it interacts with other cognitive faculties. However, I am suspicious about how much the abstract generative theory will contribute to the description of linguistic facts in a particular language. While generative theory is claimed to aim both for descriptive adequacy and for explanatory adequacy, the recent generative theory is likely to put much more weight on explanatory adequacy. In my view, a less abstract theory is enough, or even more useful, for the purpose of linguistic description. Of course, how abstract theory one should adopt as a framework differs depending upon what aspect of language one attempts to describe. What I would like to emphasize here is that linguistic theory does not conflicts with linguistic description, and a linguistic theory with an appropriate degree of abstractness serves as a tool for finding out new interesting facts, as well as for describing them in some general, elegant forms.

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