Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin
The novel gene NPHS1 is defective in the patients with congenital nephrotic syndrome of the Finnish type (CNF) leading to abnormal expression of the respective protein product nephrin in glomerular cells. CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show r...
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| Veröffentlicht in: | Experimental nephrology 2001-01, Vol.9 (5), p.327-331 |
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| creator | Wang, Shi-Xuan Ahola, Heikki Palmen, Tuula Solin, Marja-Liisa Luimula, Pauliina Holthöfer, Harry |
| description | The novel gene NPHS1 is defective in the patients with congenital nephrotic syndrome of the Finnish type (CNF) leading to abnormal expression of the respective protein product nephrin in glomerular cells. CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show recurrence of nephrotic syndrome (NS). We used indirect immunofluorescence microscopy and immunoblotting and an ELISA assay to search for circulating autoantibodies to nephrin, the protein defect in CNF patient kidneys. In serial serum samples gathered before and after recurrence of NS, we show an increased antibody titer to nephrin prior to the NS episode and a subsequent drop in antibody level after its successful treatment and reactivity of the high titer sera with glomeruli in indirect immunofluorescence microscopy as well. The results show that the transplantation treatment introduces a neoantigen inducing production of autoantibodies, which may be pathogenic for perturbation of the function of the glomerular filtration barrier. |
| doi_str_mv | 10.1159/000052628 |
| format | Article |
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CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show recurrence of nephrotic syndrome (NS). We used indirect immunofluorescence microscopy and immunoblotting and an ELISA assay to search for circulating autoantibodies to nephrin, the protein defect in CNF patient kidneys. In serial serum samples gathered before and after recurrence of NS, we show an increased antibody titer to nephrin prior to the NS episode and a subsequent drop in antibody level after its successful treatment and reactivity of the high titer sera with glomeruli in indirect immunofluorescence microscopy as well. The results show that the transplantation treatment introduces a neoantigen inducing production of autoantibodies, which may be pathogenic for perturbation of the function of the glomerular filtration barrier.</description><identifier>ISSN: 1018-7782</identifier><identifier>ISSN: 1660-2129</identifier><identifier>EISSN: 1660-2129</identifier><identifier>DOI: 10.1159/000052628</identifier><identifier>PMID: 11549850</identifier><language>eng</language><publisher>Basel, Switzerland: Karger</publisher><subject>Amino Acid Sequence ; Autoantibodies - blood ; Autoantibodies - metabolism ; Biological and medical sciences ; Child, Preschool ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Immunohistochemistry ; Infant ; Kidney - immunology ; Kidney - pathology ; Kidney Transplantation ; Male ; Medical sciences ; Membrane Proteins ; Molecular Sequence Data ; Nephrology. Urinary tract diseases ; Nephropathies. Renovascular diseases. Renal failure ; Nephrotic Syndrome - immunology ; Nephrotic Syndrome - pathology ; Nephrotic Syndrome - surgery ; Original Paper ; Proteins - chemistry ; Proteins - genetics ; Proteins - immunology ; Proteins - metabolism ; Recurrence ; Renal failure</subject><ispartof>Experimental nephrology, 2001-01, Vol.9 (5), p.327-331</ispartof><rights>2001 S. Karger AG, Basel</rights><rights>2001 INIST-CNRS</rights><rights>Copyright 2001 S. Karger AG, Basel</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c423t-cf95154fc6bd97f403b44e763a0bd74b98ae29d4bcf9fc16b8bff6353160fc3e3</citedby><cites>FETCH-LOGICAL-c423t-cf95154fc6bd97f403b44e763a0bd74b98ae29d4bcf9fc16b8bff6353160fc3e3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,2422,27903,27904</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1140098$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11549850$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Wang, Shi-Xuan</creatorcontrib><creatorcontrib>Ahola, Heikki</creatorcontrib><creatorcontrib>Palmen, Tuula</creatorcontrib><creatorcontrib>Solin, Marja-Liisa</creatorcontrib><creatorcontrib>Luimula, Pauliina</creatorcontrib><creatorcontrib>Holthöfer, Harry</creatorcontrib><title>Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin</title><title>Experimental nephrology</title><addtitle>Nephron Exp Nephrol</addtitle><description>The novel gene NPHS1 is defective in the patients with congenital nephrotic syndrome of the Finnish type (CNF) leading to abnormal expression of the respective protein product nephrin in glomerular cells. CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show recurrence of nephrotic syndrome (NS). We used indirect immunofluorescence microscopy and immunoblotting and an ELISA assay to search for circulating autoantibodies to nephrin, the protein defect in CNF patient kidneys. In serial serum samples gathered before and after recurrence of NS, we show an increased antibody titer to nephrin prior to the NS episode and a subsequent drop in antibody level after its successful treatment and reactivity of the high titer sera with glomeruli in indirect immunofluorescence microscopy as well. The results show that the transplantation treatment introduces a neoantigen inducing production of autoantibodies, which may be pathogenic for perturbation of the function of the glomerular filtration barrier.</description><subject>Amino Acid Sequence</subject><subject>Autoantibodies - blood</subject><subject>Autoantibodies - metabolism</subject><subject>Biological and medical sciences</subject><subject>Child, Preschool</subject><subject>Enzyme-Linked Immunosorbent Assay</subject><subject>Female</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Infant</subject><subject>Kidney - immunology</subject><subject>Kidney - pathology</subject><subject>Kidney Transplantation</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Membrane Proteins</subject><subject>Molecular Sequence Data</subject><subject>Nephrology. Urinary tract diseases</subject><subject>Nephropathies. Renovascular diseases. Renal failure</subject><subject>Nephrotic Syndrome - immunology</subject><subject>Nephrotic Syndrome - pathology</subject><subject>Nephrotic Syndrome - surgery</subject><subject>Original Paper</subject><subject>Proteins - chemistry</subject><subject>Proteins - genetics</subject><subject>Proteins - immunology</subject><subject>Proteins - metabolism</subject><subject>Recurrence</subject><subject>Renal failure</subject><issn>1018-7782</issn><issn>1660-2129</issn><issn>1660-2129</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNptkEtLxDAUhYMovheuBQkigotqkj7SLodhRgUZwQe4K0l6o9FOU5N2Mf_ejC3qwrvJJefLyeEgdETJJaVpcUXCpCxj-QbapVlGIkZZsRl2QvOI85ztoD3v3wlhjFKyjXbCq6TIU7KLxAOo3jloFGCr8QLaN2c7o_DjqqmcXQIWugOHn5xofFuLphOdsQ02DZ4u5vjW46oH3Fk86TsbVCNtZcCvb769THOAtrSoPRyO5z56ns-epjfR3f317XRyF6mExV2kdJGGVFplsiq4TkgskwR4FgsiK57IIhfAiiqRAdSKZjKXWmdxGtOMaBVDvI_OB9_W2c8efFcujVdQh8xge19ySjknnAfwYgCVs9470GXrzFK4VUlJue6z_OkzsCejaS-XUP2SY4EBOBsB4ZWodahJGf-HSwgp1j6nA_Yh3Cu4H332svj-qGwrHaDjf6Ehyhc-cZGs</recordid><startdate>20010101</startdate><enddate>20010101</enddate><creator>Wang, Shi-Xuan</creator><creator>Ahola, Heikki</creator><creator>Palmen, Tuula</creator><creator>Solin, Marja-Liisa</creator><creator>Luimula, Pauliina</creator><creator>Holthöfer, Harry</creator><general>Karger</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20010101</creationdate><title>Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin</title><author>Wang, Shi-Xuan ; Ahola, Heikki ; Palmen, Tuula ; Solin, Marja-Liisa ; Luimula, Pauliina ; Holthöfer, Harry</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c423t-cf95154fc6bd97f403b44e763a0bd74b98ae29d4bcf9fc16b8bff6353160fc3e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Amino Acid Sequence</topic><topic>Autoantibodies - blood</topic><topic>Autoantibodies - metabolism</topic><topic>Biological and medical sciences</topic><topic>Child, Preschool</topic><topic>Enzyme-Linked Immunosorbent Assay</topic><topic>Female</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Infant</topic><topic>Kidney - immunology</topic><topic>Kidney - pathology</topic><topic>Kidney Transplantation</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Membrane Proteins</topic><topic>Molecular Sequence Data</topic><topic>Nephrology. Urinary tract diseases</topic><topic>Nephropathies. Renovascular diseases. Renal failure</topic><topic>Nephrotic Syndrome - immunology</topic><topic>Nephrotic Syndrome - pathology</topic><topic>Nephrotic Syndrome - surgery</topic><topic>Original Paper</topic><topic>Proteins - chemistry</topic><topic>Proteins - genetics</topic><topic>Proteins - immunology</topic><topic>Proteins - metabolism</topic><topic>Recurrence</topic><topic>Renal failure</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Wang, Shi-Xuan</creatorcontrib><creatorcontrib>Ahola, Heikki</creatorcontrib><creatorcontrib>Palmen, Tuula</creatorcontrib><creatorcontrib>Solin, Marja-Liisa</creatorcontrib><creatorcontrib>Luimula, Pauliina</creatorcontrib><creatorcontrib>Holthöfer, Harry</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Experimental nephrology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Wang, Shi-Xuan</au><au>Ahola, Heikki</au><au>Palmen, Tuula</au><au>Solin, Marja-Liisa</au><au>Luimula, Pauliina</au><au>Holthöfer, Harry</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin</atitle><jtitle>Experimental nephrology</jtitle><addtitle>Nephron Exp Nephrol</addtitle><date>2001-01-01</date><risdate>2001</risdate><volume>9</volume><issue>5</issue><spage>327</spage><epage>331</epage><pages>327-331</pages><issn>1018-7782</issn><issn>1660-2129</issn><eissn>1660-2129</eissn><abstract>The novel gene NPHS1 is defective in the patients with congenital nephrotic syndrome of the Finnish type (CNF) leading to abnormal expression of the respective protein product nephrin in glomerular cells. CNF patients are treated with early nephrectomy and renal transplantation, but about 20% show recurrence of nephrotic syndrome (NS). We used indirect immunofluorescence microscopy and immunoblotting and an ELISA assay to search for circulating autoantibodies to nephrin, the protein defect in CNF patient kidneys. In serial serum samples gathered before and after recurrence of NS, we show an increased antibody titer to nephrin prior to the NS episode and a subsequent drop in antibody level after its successful treatment and reactivity of the high titer sera with glomeruli in indirect immunofluorescence microscopy as well. The results show that the transplantation treatment introduces a neoantigen inducing production of autoantibodies, which may be pathogenic for perturbation of the function of the glomerular filtration barrier.</abstract><cop>Basel, Switzerland</cop><pub>Karger</pub><pmid>11549850</pmid><doi>10.1159/000052628</doi><tpages>5</tpages></addata></record> |
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| subjects | Amino Acid Sequence Autoantibodies - blood Autoantibodies - metabolism Biological and medical sciences Child, Preschool Enzyme-Linked Immunosorbent Assay Female Humans Immunohistochemistry Infant Kidney - immunology Kidney - pathology Kidney Transplantation Male Medical sciences Membrane Proteins Molecular Sequence Data Nephrology. Urinary tract diseases Nephropathies. Renovascular diseases. Renal failure Nephrotic Syndrome - immunology Nephrotic Syndrome - pathology Nephrotic Syndrome - surgery Original Paper Proteins - chemistry Proteins - genetics Proteins - immunology Proteins - metabolism Recurrence Renal failure |
| title | Recurrence of Nephrotic Syndrome after Transplantation in CNF Is due to Autoantibodies to Nephrin |
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