Abstract:
:The liver is an active site for the biosynthesis of haem and porphyrinogens/porphyrins, which are intermediates of the haem biosynthetic pathway, because haem is required for functional activity of the cytochrome P 450 system and other critical hepatic haemoproteins. The production of hepatic haem is regulated primarily through the activity of aminolaevulinic acid synthase which is the first and normally rate-limiting enzyme of the pathway. This is, in turn, controlled by a putative regulatory haem pool. Hepatic haem can be repleted by the intravenous administration of haem, which is the basis for haem therapy in patients with acute porphyric attacks. The liver catabolizes haem to bilirubin through microsomal haem oxygenase activity and excretes haem into bile along with porphyrins. Biliary excretion of porphyrins increases significantly in patients with some types of porphyria. In protoporphyria this may cause liver damage as a result of protoporphyrin toxicity. The delineation of the pathway for protoporphyrin excretion into bile should facilitate therapy in protoporphyria by identifying ways in which protoporphyrin excretion can be enhanced.
journal_name
J Gastroenterol Hepatoljournal_title
Journal of gastroenterology and hepatologyauthors
Bloomer JRdoi
10.1111/j.1440-1746.1998.01548.xsubject
Has Abstractpub_date
1998-03-01 00:00:00pages
324-9issue
3eissn
0815-9319issn
1440-1746journal_volume
13pub_type
杂志文章,评审abstract::Autoimmune hepatitis (AIH) is an inflammatory liver disease with diverse clinical spectrum, which predominantly affects females. This review provides detailed comparisons of epidemiology, genetic predispositions, clinical features, risk factors of hepatocellular carcinoma, and mortality in AIH patients between eastern...
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pub_type: 杂志文章,随机对照试验
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pub_type: 历史文章,杂志文章,评审
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pub_type: 杂志文章
doi:10.1111/j.1440-1746.1992.tb01012.x
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,随机对照试验
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pub_type: 杂志文章
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,meta分析
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更新日期:2015-03-01 00:00:00
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pub_type: 杂志文章
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更新日期:2009-07-01 00:00:00
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.12448
更新日期:2014-04-01 00:00:00
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,评审
doi:10.1046/j.1440-1746.2000.02112.x
更新日期:2000-05-01 00:00:00
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章
doi:10.1111/jgh.13772
更新日期:2017-11-01 00:00:00
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journal_title:Journal of gastroenterology and hepatology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/j.1440-1746.2009.06151.x
更新日期:2010-05-01 00:00:00