Abstract：
:Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα-/-, Scurfy, and Ae2a,b-/- mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.
journal_name
Clin Rev Allergy Immunoljournal_title
Clinical reviews in allergy & immunologyauthors
Katsumi T,Tomita K,Leung PS,Yang GX,Gershwin ME,Ueno Ydoi
10.1007/s12016-015-8482-ysubject
Has Abstractpub_date
2015-06-01 00:00:00pages
142-53issue
2-3eissn
1080-0549issn
1559-0267journal_volume
48pub_type
杂志文章,评审abstract：:Primary biliary cirrhosis (PBC) is a rare inflammatory liver disease for which ursodeoxycholic acid (UDCA) is the only therapy approved by the U.S. Food and Drug Administration. Patients with a biochemical response to UDCA therapy have a similar survival rate compared to the general population. However, up to 40% of P...
journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-014-8456-5
更新日期：2015-06-01 00:00:00
abstract：:Strategies to improve patients' adherence to treatment are essential to reduce the great health and economic burden of allergic rhinitis and asthma. Mobile phone applications (apps) for a better management of allergic diseases are growing in number, but their usefulness for doctors and patients is still debated. Contr...
journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,meta分析,评审
doi：10.1007/s12016-016-8542-y
更新日期：2019-04-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-009-8175-5
更新日期：2010-04-01 00:00:00
abstract：:Glatiramer acetate (GA) significantly ameliorates multiple sclerosis and was initially discovered through its effects on the animal model experimental autoimmune encephalomyelitis (EAE). Guillain-Barré syndrome (GBS) is a relatively common demyelinating disease of peripheral nerves for which there is a parallel animal...
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pub_type： 杂志文章
doi：10.1007/s12016-010-8246-7
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abstract：:Reduced bone mineral density is frequently found especially in adult celiac disease (CD) and dietary guidelines favor vitamin D supplementation in adults and children with CD. Vitamin D serum levels were investigated in CD populations in order to challenge its routine supplementation. Israeli (61), Spanish (59), CD ch...
journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章
doi：10.1007/s12016-010-8237-8
更新日期：2012-06-01 00:00:00
abstract：:Adult-onset Still's disease (AOSD) is a rare multisystem autoinflammatory disorder of unknown etiology. AOSD is generally characterized by high spiking fever, arthralgia or arthritis, skin rash, leukocytosis, and hyperferritinemia. Traditionally, AOSD has been treated with non-steroidal anti-inflammatory drugs, cortic...
journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-019-08747-8
更新日期：2020-02-01 00:00:00
abstract：:Many immune-mediated diseases like inflammatory bowel disease, multiple sclerosis, type 1 diabetes, asthma, and food allergy appeared to have increased in frequency in developed countries in the latter part of the twentieth century. Reports from less developed countries suggest that the "epidemic" of immune-mediated d...
journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-014-8458-3
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/BF02737598
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pub_type： 杂志文章,评审
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更新日期：2020-08-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2012-04-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.1007/BF02826585
更新日期：1997-07-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-007-8036-z
更新日期：2008-04-01 00:00:00
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pub_type： 杂志文章,meta分析,评审
doi：10.1007/s12016-016-8531-1
更新日期：2016-12-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.1007/s12016-014-8448-5
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-020-08823-4
更新日期：2021-02-01 00:00:00
abstract：:In the vast database of peer-reviewed articles, the number of 2018 papers published retrieved using the "autoimmunity" keyword remained unchanged compared with the brilliant results of 2017 while returning above a 5% share within the immunology field, after the brisk decrease of this ratio in 2017. As in the past 12 y...
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pub_type： 杂志文章
doi：10.1007/s12016-019-08745-w
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pub_type： 杂志文章,评审
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/BF02737633
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pub_type： 杂志文章,评审
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更新日期：2019-10-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
doi：10.1007/s12016-007-8000-y
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1385/CRIAI:25:2:115
更新日期：2003-10-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
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更新日期：2018-02-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章
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更新日期：2006-10-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-017-8661-0
更新日期：2019-06-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
doi：10.1007/s12016-016-8596-x
更新日期：2017-08-01 00:00:00
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journal_title：Clinical reviews in allergy & immunology
pub_type： 杂志文章,评审
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