PCH

出典: meddic

発作性寒冷血色素尿症 paroxysmal cold hemoglobinuria

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/05/13 11:26:54」(JST)

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英文文献

  • Differential protein-protein interactions of full length human FasL and FasL fragments generated by proteolysis.
  • Lettau M1, Voss M2, Ebsen H3, Kabelitz D4, Janssen O5.Author information 1Institute of Immunology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Building 17, D-24105 Kiel, Germany. Electronic address: lettau@immunologie.uni-kiel.de.2Institute of Immunology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Building 17, D-24105 Kiel, Germany. Electronic address: Matthias.Voss@dzne.lmu.de.3Institute of Immunology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Building 17, D-24105 Kiel, Germany. Electronic address: Henriette.Oberdoerster@uksh.de.4Institute of Immunology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Building 17, D-24105 Kiel, Germany. Electronic address: Dietrich.Kabelitz@uksh.de.5Institute of Immunology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Building 17, D-24105 Kiel, Germany. Electronic address: Ottmar.Janssen@uksh.de.AbstractFas ligand (FasL) is a death factor of the tumor necrosis factor superfamily. Like other members of this family of type II transmembrane proteins, FasL is subject to ectodomain shedding by a disintegrin and metalloproteinases (ADAMs) liberating soluble FasL and leaving membrane-integral N-terminal fragments (NTFs). These NTFs are further processed by intramembrane proteolysis through signal peptide peptidase-like 2a (SPPL2a), releasing intracellular domains (ICDs) which might translocate to the nucleus to regulate transcription. Previous work established that the proline-rich domain within the cytosolic N-terminus of FasL is required for protein-protein interactions with different Src homology 3 (SH3) or WW domain proteins. Distinct binding partners regulate FasL storage and surface appearance or are involved in other aspects of FasL biology. Given the large number of FasL interactors, we asked whether proteolytically processed FasL fragments associate with the same or distinct sets of SH3 domain proteins. To address this, we performed co-precipitation experiments using a monoclonal antibody directed against the FasL N-terminus for subsequent protein detection of full length FasL and NTFs/ICDs in Western blots. We demonstrate that members of the sorting nexin (SNX) family bind full length FasL and its N-terminal fragments whereas members of the Pombe Cdc15 homology (PCH) protein family bind full length FasL, but fail to associate with processed FasL. Thus, we provide first evidence that full length FasL and FasL fragments display selectivity regarding their association with intracellular binding partners. The differential binding most likely governs the fate and function of the intracellular FasL fragments.
  • Experimental cell research.Exp Cell Res.2014 Jan 15;320(2):290-301. doi: 10.1016/j.yexcr.2013.11.016. Epub 2013 Nov 26.
  • Fas ligand (FasL) is a death factor of the tumor necrosis factor superfamily. Like other members of this family of type II transmembrane proteins, FasL is subject to ectodomain shedding by a disintegrin and metalloproteinases (ADAMs) liberating soluble FasL and leaving membrane-integral N-terminal f
  • PMID 24291222
  • A mutation associated with centronuclear myopathy enhances the size and stability of dynamin 2 complexes in cells.
  • James NG, Digman MA, Ross JA, Barylko B, Wang L, Li J, Chen Y, Mueller JD, Gratton E, Albanesi JP, Jameson DM.Author information Department of Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, 651 Ilalo Street, Biosciences 222, Honolulu, HI 96813, USA.AbstractBACKGROUND: Dynamin 2 (Dyn2) is a ~100kDa GTPase that assembles around the necks of nascent endocytic and Golgi vesicles and catalyzes membrane scission. Mutations in Dyn2 that cause centronuclear myopathy (CNM) have been shown to stabilize Dyn2 polymers against GTP-dependent disassembly in vitro. Precisely timed regulation of assembly and disassembly is believed to be critical for Dyn2 function in membrane vesiculation, and the CNM mutations interfere with this regulation by shifting the equilibrium toward the assembled state.
  • Biochimica et biophysica acta.Biochim Biophys Acta.2014 Jan;1840(1):315-21. doi: 10.1016/j.bbagen.2013.09.001. Epub 2013 Sep 7.
  • BACKGROUND: Dynamin 2 (Dyn2) is a ~100kDa GTPase that assembles around the necks of nascent endocytic and Golgi vesicles and catalyzes membrane scission. Mutations in Dyn2 that cause centronuclear myopathy (CNM) have been shown to stabilize Dyn2 polymers against GTP-dependent disassembly in vitro. P
  • PMID 24016602
  • Presumed primary and secondary hepatic copper accumulation in cats.
  • Hurwitz BM, Center SA, Randolph JF, McDonough SP, Warner KL, Hazelwood KS, Chiapella AM, Mazzei MJ, Leavey K, Acquaviva AE, Lindsay MM, Sanders L, Pintar J.Author information Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853.AbstractObjective-To determine signalments, clinical features, clinicopathologic variables, imaging findings, treatments, and survival time of cats with presumed primary copper-associated hepatopathy (PCH) and to determine quantitative measures and histologic characteristics of the accumulation and distribution of copper in liver samples of cats with presumed PCH, extrahepatic bile duct obstruction, chronic nonsuppurative cholangitis-cholangiohepatitis, and miscellaneous other hepatobiliary disorders and liver samples of cats without hepatobiliary disease. Design-Retrospective cross-sectional study. Animals-100 cats with hepatobiliary disease (PCH [n = 11], extrahepatic bile duct obstruction [14], cholangitis-cholangiohepatitis [37], and miscellaneous hepatobiliary disorders [38]) and 14 cats without hepatobiliary disease. Procedures-From 1980 to 2013, cats with and without hepatobiliary disease confirmed by liver biopsy and measurement of hepatic copper concentrations were identified. Clinical, clinicopathologic, and imaging data were compared between cats with and without PCH. Results-Cats with PCH were typically young (median age, 2.0 years); clinicopathologic and imaging characteristics were similar to those of cats with other liver disorders. Copper-specific staining patterns and quantification of copper in liver samples confirmed PCH (on the basis of detection of > 700 μg/g of liver sample dry weight). Six cats with PCH underwent successful treatment with chelation (penicillamine; n = 5), antioxidants (5), low doses of elemental zinc (2), and feeding of hepatic support or high-protein, low-carbohydrate diets, and other hepatic support treatments. One cat that received penicillamine developed hemolytic anemia, which resolved after discontinuation of administration. Three cats with high hepatic copper concentrations developed hepatocellular neoplasia. Conclusions and Clinical Relevance-Results suggested that copper accumulates in livers of cats as primary and secondary processes. Long-term management of cats with PCH was possible.
  • Journal of the American Veterinary Medical Association.J Am Vet Med Assoc.2014 Jan 1;244(1):68-77. doi: 10.2460/javma.244.1.68.
  • Objective-To determine signalments, clinical features, clinicopathologic variables, imaging findings, treatments, and survival time of cats with presumed primary copper-associated hepatopathy (PCH) and to determine quantitative measures and histologic characteristics of the accumulation and distribu
  • PMID 24344855

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★リンクテーブル★
リンク元溶血性貧血」「paroxysmal cold hemoglobinuria
拡張検索GTPCHI
関連記事PC」「P

溶血性貧血」

  [★]

hemolytic anemia
溶血性黄疸 hemolytic jaundice
貧血
溶血の種類 機序 脾腫 ヘモグロビン尿
ヘモジデリン尿
疾患
血管外溶血 Mφで貪食される 球状赤血球症 HS
自己免疫性溶血性貧血 AIHA (温式)
↑冷式より多い
血管内溶血 血管内で溶血 自己免疫性溶血性貧血 AIHA (冷式)
発作性夜間ヘモグロビン尿症 PNH
G6PD欠損症
赤血球破砕症候群
不適合輸血

概念

  • 赤血球寿命が種々の原因により短縮することに伴う貧血の総称。

病因

  • 赤血球が何らかの原因により破壊され、骨髄の代償能力を超えて赤血球が減少することにより貧血を呈する。 → 溶血が存在するが貧血ではない状態がある。
  • 溶血の原因
  • 先天性疾患
  • 後天性疾患
  • 自己抗体による溶血

HIM.653

赤血球内の素因

  • 1. ヘモグロビンの異常
  • 2. 膜-細胞骨格複合体の異常
  • 3. 代謝経路

赤血球外の素因

  • 先天性
  • 家族性溶血尿毒症 familial hemolytic uremic syndrome
  • 後天性

症状

  • 貧血、無胆汁色素尿性または溶血性黄疸、脾腫

参考

  • [charged]Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and hypersplenism - uptodate
[display]http://www.uptodate.com/contents/extrinsic-nonimmune-hemolytic-anemia-due-to-mechanical-damage-fragmentation-hemolysis-and-hypersplenism?source=search_result&selectedTitle=2%7E150



paroxysmal cold hemoglobinuria」

  [★] 発作性寒冷血色素尿症 PCH


GTPCHI」

  [★]

GTP cyclohydrolase I


PC」

  [★]

PrepTutorEJDIC   license prepejdic

「Peace Corps 平和部隊」


P」

  [★]


WordNet   license wordnet

「the 16th letter of the Roman alphabet」
p

PrepTutorEJDIC   license prepejdic

「parking」

PrepTutorEJDIC   license prepejdic

「phosphorusの化学記号」


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