Abstract:
:Ferritin, composed of H-subunits and L-subunits, plays important roles in iron storage and in the control of intracellular iron distribution. Synthesis of both subunits is controlled by common cytoplasmic proteins, iron regulatory proteins (IRP-1 and IRP-2) that bind to the iron-responsive element (IRE) in the 5'-untranslated region of ferritin messenger RNA (mRNA). When intracellular iron is scarce, IRPs display IRE binding to suppress translation of mRNA. When cellular iron is abundant, IRPs become inactivated (IRP-1) or degraded (IRP-2). In the last few years, IRE mutations that cause disorders due to dysregulation of ferritin subunit synthesis have been identified. Hereditary hyperferritinemia-cataract syndrome is associated with point mutations or deletions in the IRE of L-subunit mRNA and is characterized by constitutively increased synthesis of L-subunits but is unrelated to iron overload. A single-point mutation in the IRE of H-subunit mRNA in members of a family affected with dominantly inherited iron overload has been reported. This review summarizes the current understanding of the translational disorders caused by IRE mutations in ferritin mRNA.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Kato J,Niitsu Ydoi
10.1007/BF02982789subject
Has Abstractpub_date
2002-10-01 00:00:00pages
208-12issue
3eissn
0925-5710issn
1865-3774journal_volume
76pub_type
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journal_title:International journal of hematology
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journal_title:International journal of hematology
pub_type: 临床试验,杂志文章
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abstract::The author would like to correct the error in the "Abstract" section of original publication as given below. ...
journal_title:International journal of hematology
pub_type: 杂志文章,已发布勘误
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pub_type: 临床试验,杂志文章
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pub_type: 杂志文章,评审
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更新日期:2013-09-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-013-1350-8
更新日期:2013-07-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1211-x
更新日期:2012-12-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章,评审
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journal_title:International journal of hematology
pub_type: 杂志文章
doi:
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pub_type: 杂志文章,评审
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