Abstract:
:Abetalipoproteinemia is a recessive genetic disease in humans characterized by the virtual absence of apolipoprotein (apo)B and apoB-containing lipoproteins in plasma. Microsomal triglyceride transfer protein (MTP), a resident lipid transfer protein within the endoplasmic reticulum of hepatocytes and enterocytes, has been shown to be absent in enterocytes from subjects with this disease. MTP is a heterodimer of a unique large subunit and protein disulfide isomerase. It has been demonstrated that the absence of MTP in abetalipoproteinemia is secondary to mutations in the gene for the large subunit of MTP. Thus, mutations in the gene for the large subunit of MTP are a cause of abetalipoproteinemia, which indicates that the MTP is a necessary component for the assembly and secretion of apoB-containing lipoproteins from the liver and intestine.
journal_name
Curr Opin Lipidoljournal_title
Current opinion in lipidologyauthors
Gregg RE,Wetterau JRdoi
10.1097/00041433-199404000-00003subject
Has Abstractpub_date
1994-04-01 00:00:00pages
81-6issue
2eissn
0957-9672issn
1473-6535journal_volume
5pub_type
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
doi:10.1097/00041433-199408000-00007
更新日期:1994-08-01 00:00:00
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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更新日期:2013-10-01 00:00:00
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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更新日期:2013-06-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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更新日期:1995-12-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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更新日期:2007-06-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
doi:10.1097/MOL.0b013e32833854ac
更新日期:2010-06-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
doi:10.1097/00041433-200008000-00007
更新日期:2000-08-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
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更新日期:1999-12-01 00:00:00
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journal_title:Current opinion in lipidology
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更新日期:2018-10-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
doi:10.1097/00041433-199804000-00009
更新日期:1998-04-01 00:00:00
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journal_title:Current opinion in lipidology
pub_type: 杂志文章,评审
doi:10.1097/00041433-199604000-00006
更新日期:1996-04-01 00:00:00
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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更新日期:2004-06-01 00:00:00
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更新日期:2009-02-01 00:00:00
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journal_title:Current opinion in lipidology
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更新日期:2015-04-01 00:00:00
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更新日期:2002-10-01 00:00:00
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journal_title:Current opinion in lipidology
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更新日期:2007-10-01 00:00:00
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journal_title:Current opinion in lipidology
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journal_title:Current opinion in lipidology
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更新日期:2016-12-01 00:00:00
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journal_title:Current opinion in lipidology
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更新日期:2005-10-01 00:00:00