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)[Wiki en表示]
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- 1. 成人における慢性膵炎の臨床症状および診断 clinical manifestations and diagnosis of chronic pancreatitis in adults
- 2. 吸収不良の治療の概要 overview of the treatment of malabsorption
- 3. 嚢胞性線維症：膵機能不全の評価およびマネージメント cystic fibrosis assessment and management of pancreatic insufficiency
- 4. 膵外分泌機能検査 pancreatic exocrine function tests
- 5. Cystic fibrosis-related diabetes mellitus
- 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
- 東京女子医科大学雑誌 85(4), 125-130, 2015-08-25
- NAID 110009931211
- 日本臨床外科学会雑誌 76(2), 374-381, 2015
- NAID 130005095228
- Liraglutide Activates AMPK Signaling and Partially Restores Normal Circadian Rhythm and Insulin Secretion in Pancreatic Islets in Diabetic Mice
- Biological and Pharmaceutical Bulletin 38(8), 1142-1149, 2015
- NAID 130005090636
- 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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