Abstract：
BACKGROUND:The characteristic feature of Goodpasture disease is the occurrence of an autoantibody response to the noncollagenous domain of the alpha3 chain of type IV collagen [alpha3(IV)NC1] in the alveolar and glomerular basement membrane. These antibodies are associated with the development of a rapidly progressive glomerulonephritis, with or without lung hemorrhage, whereas autoantibodies specific for the other alpha chains of the heterotrimeric type IV collagen probably do not cause disease. In this study, we have investigated whether differences in fine specificity of autoimmune recognition of the alpha3(IV)NC1 correlate with clinical outcome. METHODS:For mapping of antibody binding to type IV collagen, chimeric collagen constructs were generated in which parts of the alpha3(IV)NC1 domain were replaced by the corresponding sequences of homologous nonreactive alpha1(IV). The different recombinant collagen chimeras allowed the analysis of antibody specificities in 77 sera from well-documented patients. RESULTS:One construct that harbors the aminoterminal third of the alpha3(IV)NC1 was recognized by all sera, indicating that it represents the dominant target of the B-cell response in Goodpasture disease. Seventy percent of the samples recognized other parts of the molecule as well. However, only reactivity to the N-terminus of the alpha3(IV)NC1 correlated with prognosis, that is, kidney survival after six months of follow-up. CONCLUSION:The results indicate the crucial importance of antibody recognition of this particular domain for the pathogenesis of Goodpasture disease, thereby opening new avenues for the development of better diagnostic and therapeutic procedures.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Hellmark T,Segelmark M,Unger C,Burkhardt H,Saus J,Wieslander Jdoi
10.1046/j.1523-1755.1999.055003936.xsubject
Has Abstractpub_date
1999-03-01 00:00:00pages
936-44issue
3eissn
0085-2538issn
1523-1755pii
S0085-2538(15)46040-5journal_volume
55pub_type
杂志文章abstract：:Defective recycling of megalin consequent to impaired phosphatidylinositol metabolism has been implicated as a mechanism causing tubular proteinuria in patients with Lowe syndrome. In this issue, Berquez et al. describe an innovative approach using a clinically approved drug to "rebalance" phosphatidylinositols and re...
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abstract：BACKGROUND:The "intact" parathyroid hormone (PTH) assay recognizes PTH-(1-84) as well as amino terminally truncated PTH fragments, that is, large carboxyterminal PTH fragments (C-PTH fragments). The present study investigated whether the use of the plasma PTH-(1-84)/C-PTH fragment ratio enhances the noninvasive assessm...
journal_title：Kidney international
pub_type： 杂志文章
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abstract：:Autologous and allogeneic bone marrow grafting both require cytoreductive therapy but only the allogeneic procedure requires immunosuppressive agents. Allogeneic bone marrow transplantation has been reported to be associated with a high incidence of both renal failure and veno-occlusive disease (VOD) of the liver, the...
journal_title：Kidney international
pub_type： 临床试验,杂志文章
doi：10.1038/ki.1996.405
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abstract：:IgA nephropathy is the most common primary glomerulonephritis worldwide. Its frequent coexistence with inflammatory, infectious, or malignant processes raises the possibility of a pathologic rather than coincidental association. Major strides have been made to elucidate the underlying pathophysiologic events that culm...
journal_title：Kidney international
pub_type： 杂志文章,评审
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abstract：BACKGROUND:Macrophage (Mphi) infiltration may contribute to chronic renal injury. We therefore sought to examine the expression of genes associated with Mphi recruitment in the rat remnant kidney model. METHODS:Male Munich Wistar rats underwent 5/6 nephrectomy or sham operation (SHM, N = 18) and received no treatment ...
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pub_type： 杂志文章
doi：10.1111/j.1523-1755.2000.00327.x
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1992.431
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journal_title：Kidney international
pub_type： 临床试验,杂志文章,随机对照试验
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journal_title：Kidney international
pub_type： 杂志文章
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更新日期：1981-04-01 00:00:00
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1977.49
更新日期：1977-05-01 00:00:00
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pub_type： 信件
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journal_title：Kidney international
pub_type： 杂志文章
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journal_title：Kidney international
pub_type： 杂志文章
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更新日期：2006-01-01 00:00:00
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journal_title：Kidney international
pub_type： 临床试验,杂志文章
doi：10.1038/ki.1994.378
更新日期：1994-10-01 00:00:00
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1985.42
更新日期：1985-03-01 00:00:00
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journal_title：Kidney international
pub_type： 杂志文章,评审
doi：10.1046/j.1523-1755.1999.00610.x
更新日期：1999-09-01 00:00:00
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.2011.42
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abstract：:Kinetic parameters were studied to determine the effectiveness of hemoperfusion in removing ethychlorvynol from the plasma and red blood cells (RBC) of intoxicated dogs. Perfusion columns contained polystyrene/divinyl benzene resin (XAD-4 Amberlite. Column clearances of ethchlorvynol averaged 96.5 +/- 0.4% of the plas...
journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1980.36
更新日期：1980-03-01 00:00:00
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journal_title：Kidney international
pub_type： 评论,杂志文章
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journal_title：Kidney international
pub_type： 杂志文章
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journal_title：Kidney international
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1046/j.1523-1755.1999.00235.x
更新日期：1999-01-01 00:00:00
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journal_title：Kidney international
pub_type： 杂志文章
doi：10.1038/ki.1997.317
更新日期：1997-07-01 00:00:00
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pub_type： 杂志文章
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journal_title：Kidney international
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journal_title：Kidney international
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journal_title：Kidney international
pub_type： 杂志文章
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更新日期：1995-09-01 00:00:00
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journal_title：Kidney international
pub_type： 评论,杂志文章
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doi：10.1038/ki.2012.347
更新日期：2013-03-01 00:00:00
