pancreatic insufficiency

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2014/04/07 03:11:19」(JST)

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英文文献

  • Stable activity of diabetogenic cells with age in NOD mice: dynamics of reconstitution and adoptive diabetes transfer in immunocompromized mice.
  • Kaminitz A1, Mizrahi K, Ash S, Ben-Nun A, Askenasy N.Author information 1Frankel Laboratory, Center for Stem Cell Research, Bone Marrow Transplant Unit, Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, 49202; Department of Immunology, The Weizmann Institute of Science, Rehovot, 76100, Israel.AbstractThe NOD mouse is a prevalent disease model of type 1 diabetes. Immune aberrations that cause and propagate autoimmune insulitis in these mice are being continuously debated, with evidence supporting both dominance of effector cells and insufficiency of suppressor mechanisms. In this study we assessed the behavior of NOD lymphocytes under extreme expansion conditions using adoptive transfer into immunocompromized NOD.SCID mice. CD4+ CD25+ T cells do not cause islet inflammation, whereas splenocytes and CD4+ CD25- T cells induce pancreatic inflammation and hyperglycemia in 80-100% of the NOD.SCID recipients. Adoptively transferred effector T cells migrate to the lymphoid organs and pancreas, proliferate, are activated in the target organ in situ and initiate inflammatory insulitis. Reconstitution of all components of the CD4+ subset emphasizes the plastic capacity of different cell types to adopt effector and suppressor phenotyes. Furthermore, similar immune profiles of diabetic and euglycemic NOD.SCID recipients demonstrate dissociation between fractional expression of CD25 and FoxP3 and the severity of insulitis. There were no evident and consistent differences in diabetogenic activity and immune reconstituting activity of T cells from prediabetic (11 weeks) and new onset diabetic NOD females. Similarities in immune phenotypes and variable distribution of effector and suppressor subsets in various stages of inflammation commend caution in interpretation of quantitative and qualitative aberrations as markers of disease severity in adoptive transfer experiments. This article is protected by copyright. All rights reserved.
  • Immunology.Immunology.2014 Mar 7. doi: 10.1111/imm.12277. [Epub ahead of print]
  • The NOD mouse is a prevalent disease model of type 1 diabetes. Immune aberrations that cause and propagate autoimmune insulitis in these mice are being continuously debated, with evidence supporting both dominance of effector cells and insufficiency of suppressor mechanisms. In this study we assesse
  • PMID 24601987
  • Mutations in the Human UBR1 Gene and the Associated Phenotypic Spectrum.
  • Sukalo M1, Fiedler A, Guzmán C, Spranger S, Addor MC, McHeik JN, Benavent MO, Cobben JM, Gillis LA, Shealy AG, Deshpande C, Bozorgmehr B, Everman DB, Stattin EL, Liebelt J, Keller KM, Bertola DR, van Karnebeek CD, Bergmann C, Liu Z, Düker G, Rezaei N, Alkuraya FS, Oğur G, Alrajoudi A, Venegas-Vega CA, Verbeek NE, Richmond EJ, Kirbiyik O, Ranganath P, Singh A, Godbole K, Ali FA, Alves C, Mayerle J, Lerch MM, Witt H, Zenker M.Author information 1Institute of Human Genetics, University Hospital Magdeburg, Magdeburg, Germany.AbstractJohanson-Blizzard syndrome (JBS) is a rare, autosomal recessive disorder characterised by exocrine pancreatic insufficiency, typical facial features, dental anomalies, hypothyroidism, sensorineural hearing loss, scalp defects, urogenital and anorectal anomalies, short stature, and cognitive impairment of variable degree. This syndrome is caused by a defect of the E3 ubiquitin ligase UBR1, which is part of the proteolytic N-end rule pathway. Herein we review previously reported (n = 29) and a total of 31 novel UBR1 mutations in relation to the associated phenotype in patients from 50 unrelated families. Mutation types include nonsense, frameshift, splice site, missense and small in-frame deletions consistent with the hypothesis that loss of UBR1 protein function is the molecular basis of JBS. There is an association of missense mutations and small in-frame deletions with milder physical abnormalities and a normal intellectual capacity, thus suggesting that at least some of these may represent hypomorphic UBR1 alleles. The review of clinical data of a large number of molecularly confirmed JBS cases allows us to define minimal clinical criteria for the diagnosis of JBS. For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. This article is protected by copyright. All rights reserved.
  • Human mutation.Hum Mutat.2014 Mar 5. doi: 10.1002/humu.22538. [Epub ahead of print]
  • Johanson-Blizzard syndrome (JBS) is a rare, autosomal recessive disorder characterised by exocrine pancreatic insufficiency, typical facial features, dental anomalies, hypothyroidism, sensorineural hearing loss, scalp defects, urogenital and anorectal anomalies, short stature, and cognitive impairme
  • PMID 24599544

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関連リンク

Overview of pancreatic insufficiency and tests used to help detect it ... The review date indicates when the article was last reviewed from beginning to end to ensure that it reflects the most current science. A review may not require ...
pancreatic insufficiency, a condition characterized by inadequate production and secretion of pancreatic hormones or enzymes. It usually occurs secondary to a disease process destructive to pancreatic tissue. Nutritional ...

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★リンクテーブル★
リンク元膵不全」「膵機能不全
拡張検索exocrine pancreatic insufficiency
関連記事insufficiency」「pancreatic

膵不全」

  [★]

pancreatic insufficiency
膵機能不全膵外分泌不全

膵機能不全」

  [★]

pancreatic insufficiency
膵不全


exocrine pancreatic insufficiency」

  [★]

膵外分泌不全

pancreatic insufficiency

insufficiency」

  [★]

  • n.
dearthdeficientdeficitdysfunctionfailingfailurehypofunctionimperfectain shortinadequateincompetenceinsufficientinsufficientlylacklowmalfunctionpaucitypoorlyregurgitationscantyscarcescarcityshortshortageshortnessstun

WordNet   license wordnet

「a lack of competence; "pointed out the insufficiencies in my report"; "juvenile offenses often reflect an inadequacy in the parents"」
inadequacy

WordNet   license wordnet

「(pathology) inability of a bodily part or organ to function normally」

WordNet   license wordnet

「lack of an adequate quantity or number; "the inadequacy of unemployment benefits"」
inadequacy, deficiency

PrepTutorEJDIC   license prepejdic

「不十分な[点],不足;不適当,不向き」


pancreatic」

  [★]

  • adj.
  • 膵臓の、膵性の、膵の
pancreaspancreata

WordNet   license wordnet

「of or involving the pancreas; "pancreatic cancer"」

PrepTutorEJDIC   license prepejdic

「膵臓の」




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