出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/09/19 13:05:29」(JST)[Wiki en表示]
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- 1. Hereditary angioedema with normal C1 inhibitor (Type III HAE)
- 2. 後天性C1インヒビター欠損症：臨床症状、疫学、病因、および診断 acquired c1 inhibitor deficiency clinical manifestations epidemiology pathogenesis and diagnosis
- 3. 遺伝性血管浮腫：発作の予防 hereditary angioedema prevention of attacks
- 4. 遺伝性血管浮腫：病因および診断 hereditary angioedema pathogenesis and diagnosis
- 5. ACE 阻害薬誘発性血管浮腫 ace inhibitor induced angioedema
- Scube2 enhances proteolytic Shh processing from the surface of Shh-producing cells.
- Jakobs P1, Exner S, Schürmann S, Pickhinke U, Bandari S, Ortmann C, Kupich S, Schulz P, Hansen U, Seidler DG, Grobe K.Author information 1The Institute for Physiological Chemistry and Pathobiochemistry, Westfälische Wilhelms Universität Münster, Waldeyerstrasse 15, D-48149 Münster, Germany.AbstractAll morphogens of the Hedgehog (Hh) family are synthesized as dual-lipidated proteins, which results in their firm attachment to the surface of the cell in which they were produced. Thus, Hh release into the extracellular space requires accessory protein activities. We suggested previously that the proteolytic removal of N- and C-terminal lipidated peptides (shedding) could be one such activity. More recently, the secreted glycoprotein Scube2 (signal peptide, cubulin domain, epidermal-growth-factor-like protein 2) was also implicated in the release of Shh from the cell membrane. This activity strictly depended on the CUB domains of Scube2, which derive their name from the complement serine proteases and from bone morphogenetic protein-1/tolloid metalloproteinases (C1r/C1s, Uegf and Bmp1). CUB domains function as regulators of proteolytic activity in these proteins. This suggested that sheddases and Scube2 might cooperate in Shh release. Here, we confirm that sheddases and Scube2 act cooperatively to increase the pool of soluble bioactive Shh, and that Scube2-dependent morphogen release is unequivocally linked to the proteolytic processing of lipidated Shh termini, resulting in truncated soluble Shh. Thus, Scube2 proteins act as protease enhancers in this setting, revealing newly identified Scube2 functions in Hh signaling regulation.
- Journal of cell science.J Cell Sci.2014 Apr 15;127(Pt 8):1726-37. doi: 10.1242/jcs.137695. Epub 2014 Feb 12.
- All morphogens of the Hedgehog (Hh) family are synthesized as dual-lipidated proteins, which results in their firm attachment to the surface of the cell in which they were produced. Thus, Hh release into the extracellular space requires accessory protein activities. We suggested previously that the
- PMID 24522195
- Possibility of graphene growth by close space sublimation.
- Sopinskyy MV1, Khomchenko VS, Strelchuk VV, Nikolenko AS, Olchovyk GP, Vishnyak VV, Stonis VV.Author information 1V, Ye, Lashkaryov Institute of Semiconductor Physics, National Academy of Sciences of Ukraine, 45 Prospect Nauky, Kyiv 03028, Ukraine. firstname.lastname@example.org.AbstractCarbon films on the Si/SiO2 substrate are fabricated using modified method of close space sublimation at atmospheric pressure. The film properties have been characterized by micro-Raman and X-ray photoelectron spectroscopy and monochromatic ellipsometry methods. Ellipsometrical measurements demonstrated an increase of the silicon oxide film thickness in the course of manufacturing process. The XPS survey spectra of the as-prepared samples indicate that the main elements in the near-surface region are carbon, silicon, and oxygen. The narrow-scan spectra of C1s, Si2p, O1s regions indicate that silicon and oxygen are mainly in the SiOx (x ≈ 2) oxide form, whereas the main component of C1s spectrum at 284.4 eV comes from the sp2-hybridized carbon phase. Micro-Raman spectra confirmed the formation of graphene films with the number of layers that depended on the distance between the graphite source and substrate.
- Nanoscale research letters.Nanoscale Res Lett.2014 Apr 14;9(1):182. doi: 10.1186/1556-276X-9-182.
- Carbon films on the Si/SiO2 substrate are fabricated using modified method of close space sublimation at atmospheric pressure. The film properties have been characterized by micro-Raman and X-ray photoelectron spectroscopy and monochromatic ellipsometry methods. Ellipsometrical measurements demonstr
- PMID 24731549
- TNT003, an inhibitor of the serine protease C1s, prevents complement activation induced by cold agglutinin disease patient autoantibodies.
- Shi J1, Rose EL, Singh A, Hussain S, Stagliano NE, Parry GC, Panicker S.Author information 1True North Therapeutics, Inc., South San Francisco, CA, United States.AbstractActivation of the classical pathway of complement (CP) is often associated with autoimmune disorders in which disease pathology is linked to the presence of an autoantibody. One such disorder is cold agglutinin disease (CAD), an autoimmune hemolytic anemia in which autoantibodies (cold agglutinins) bind to red blood cells (RBCs) at low temperatures. Anemia occurs as a result of autoantibody mediated CP activation on the surface of the erythrocyte, leading to the deposition of complement opsonins that drive extravascular hemolysis in the liver. Here we test the effects of TNT003, a mouse monoclonal antibody targeting the CP specific serine protease C1s, on CP activity induced by cold agglutinins on human RBCs. We collected 40 individual CAD patient samples and showed that TNT003 prevented cold agglutinin mediated deposition of complement opsonins that promote phagocytosis of RBCs. Furthermore, by preventing CP activation, we show TNT003 also prevents cold agglutinin driven generation of anaphylatoxins. Finally, we provide evidence that CP activity in CAD patients terminates prior to activation of the terminal cascade, supporting the hypothesis that the primary route of RBC destruction in these patients occurs via extravascular hemolysis. Our results support the development of a CP inhibitor for the treatment of CAD.
- Blood.Blood.2014 Apr 2. [Epub ahead of print]
- Activation of the classical pathway of complement (CP) is often associated with autoimmune disorders in which disease pathology is linked to the presence of an autoantibody. One such disorder is cold agglutinin disease (CAD), an autoimmune hemolytic anemia in which autoantibodies (cold agglutinins)
- PMID 24695853
- 炭素 2015(269), 181-189, 2015
- NAID 130005100945
- スパッタリング法で調製した炭素-白金電極触媒のメタノール酸化反応選択性に及ぼすポストアニールの 影響
- Electrochemistry 83(5), 368-371, 2015
- NAID 130005067096
- Journal of the Vacuum Society of Japan 57(7), 266-271, 2014
- NAID 130004679102
|classical pathway||antigen-antibody complex||C1q, C1r, C1s, C4, C2|
|lectin pathway||mannose-binding lectin or ficolin binds carbohydrate on pathogen surface||MBL/ficolin, MASP-2, C4, C2|
|alternative pathway||pathogen surface||C3, B, D|
３つの経路は共通してC3 convertaseを生成。C3 convertaseはC3→C3a+C3b C3a: C3a is a peptide mediator of local inflammation C3b: C3b binds covalently to the bacterial cell membrane and opsonizes the bacteria
C5 convertaseはC3bにC3 convertaseが結合してできる C5 converaseはC5→C5a+C5b C5a: powerful peptide mediator of inflammation C5b: C5b,C6,C7,C8,C9: membrane-attack complex
- C5a,C3a,C4a: smooth muscle contraction.(IMM.75)
- C5a,C3a: act on the endo thelial cells lining blood vessels to induce adhesion molecules.(IMM.75)
- C5a>C3a>C4a: increase in blood flow, increase vascular permeability, increase binding of phagocytes to exdothielial cells.(IMM.76)
- C5a: activates mast cells to release mediators, such as histamine and TNF-α. that contribute the inflammatory response(IMM.76).
- C5a: acts directly on neutrophils and monocytes and attracting neutrophils and monocytes(IMM.75)
- C3a: contributes to the pypotension and edema seen in endotoxic shock
- C5a: activated by endotoxin, funcions in neutrophil chemotaxis
- C1s deficiency
- 炭素 carbon
- シトシン cytosine
- システイン cysteine
- セルシウス度 摂氏 degrees Celsius degree C
- 産婦人科領域で「子宮外頚 子宮外頚部 exocervix」を表現するための略語