WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis
Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is cause...
Gespeichert in:
Veröffentlicht in: | The American journal of pathology 1999, Vol.154 (1), p.181-192 |
---|---|
Hauptverfasser: | , , , , , |
Format: | Artikel |
Sprache: | eng |
Schlagworte: | |
Online-Zugang: | Volltext |
Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
container_end_page | 192 |
---|---|
container_issue | 1 |
container_start_page | 181 |
container_title | The American journal of pathology |
container_volume | 154 |
creator | Yang, Youxin Jeanpierre, Cécile Dressler, Gregory R. Lacoste, Mireille Niaudet, Patrick Gubler, Marie-Claire |
description | Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is caused by dominant negative point mutations in the
WT1 gene that encodes a tumor suppressor transcription factor normally expressed in podocytes. Mutations usually affect the zinc fingers of the WT1 protein. The basic defect is unknown in most cases of isolated diffuse mesangial sclerosis, a disease characterized by the same glomerular changes as in Denys-Drash syndrome but possibly transmitted as an autosomal recessive trait. Here we show that the distribution of WT1 is abnormal in most patients with Denys-Drash syndrome: WT1 nuclear staining of podocytes is decreased or absent. This finding is consistent with the decreased DNA binding capacity of the mutated protein. One target gene of WT1 is
PAX2, the expression of which is down-regulated in podocytes during early stages of nephrogenesis. We demonstrate that WT1 mislocalization is associated with abnormal podocyte expression of PAX2 protein and RNA. We suggest that persistent expression of PAX2 is likely to result from the loss of WT1 dependent transcriptional repression and may participate in the pathological mechanisms leading to glomerular dysfunction. Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in
WT1 could also be operative in isolated diffuse mesangial sclerosis. Primary involvement of
PAX2 is an alternative hypothesis because persistent expression of PAX2 in transgenic mice is associated with the occurrence of early and severe glomerulopathy. |
doi_str_mv | 10.1016/S0002-9440(10)65264-9 |
format | Article |
fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1853439</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0002944010652649</els_id><sourcerecordid>69559670</sourcerecordid><originalsourceid>FETCH-LOGICAL-c667t-2503291d91313d5d8bae2f47b2258ee9062a797788c399a78d3216446967a2863</originalsourceid><addsrcrecordid>eNqFkU9vEzEQxVcIVELhI1RaIYTgsODxrr32BVQ1BSoVUSlFcLMcezZxtLGDvSnk2-P8UQpcOFn2_ObNPL-iOAPyBgjwtxNCCK1k05BXQF5zRnlTyQfFCBhlFQUJD4vREXlcPElpka-8FuSkOJESuKzpqJh-u4VSe1venH-vaHkTbDCbAcvLX6uIKbngS-fLMfpNqsZRp3k52XgbwxJ3XVcp9HpAW45d160Tlp8xaT9zui8npscYkktPi0ed7hM-O5ynxdcPl7cXn6rrLx-vLs6vK8N5O1SUkZpKsBJqqC2zYqqRdk07pZQJREk41a1sWyFMLaVuha0p8KbhkreaCl6fFu_2uqv1dInWoB-i7tUquqWOGxW0U39XvJurWbhTIFjd1DILvDwIxPBjjWlQS5cM9r32GNZJcclYHkYy-PwfcBHW0WdzioKQQkIrMsT2kMm_kCJ2x02AqG2Capeg2sazfdolqLZbnP1p49h1iCzXXxzqOhndd1F749K9OG-gJuzezNzN5j9dRJWWuu-zKCi9WAFrFGTnkMH3exBzNncOo0rGoTdoc5MZlA3uPxv_BkWvwuE</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>218989178</pqid></control><display><type>article</type><title>WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis</title><source>MEDLINE</source><source>Access via ScienceDirect (Elsevier)</source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><creator>Yang, Youxin ; Jeanpierre, Cécile ; Dressler, Gregory R. ; Lacoste, Mireille ; Niaudet, Patrick ; Gubler, Marie-Claire</creator><creatorcontrib>Yang, Youxin ; Jeanpierre, Cécile ; Dressler, Gregory R. ; Lacoste, Mireille ; Niaudet, Patrick ; Gubler, Marie-Claire</creatorcontrib><description>Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is caused by dominant negative point mutations in the
WT1 gene that encodes a tumor suppressor transcription factor normally expressed in podocytes. Mutations usually affect the zinc fingers of the WT1 protein. The basic defect is unknown in most cases of isolated diffuse mesangial sclerosis, a disease characterized by the same glomerular changes as in Denys-Drash syndrome but possibly transmitted as an autosomal recessive trait. Here we show that the distribution of WT1 is abnormal in most patients with Denys-Drash syndrome: WT1 nuclear staining of podocytes is decreased or absent. This finding is consistent with the decreased DNA binding capacity of the mutated protein. One target gene of WT1 is
PAX2, the expression of which is down-regulated in podocytes during early stages of nephrogenesis. We demonstrate that WT1 mislocalization is associated with abnormal podocyte expression of PAX2 protein and RNA. We suggest that persistent expression of PAX2 is likely to result from the loss of WT1 dependent transcriptional repression and may participate in the pathological mechanisms leading to glomerular dysfunction. Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in
WT1 could also be operative in isolated diffuse mesangial sclerosis. Primary involvement of
PAX2 is an alternative hypothesis because persistent expression of PAX2 in transgenic mice is associated with the occurrence of early and severe glomerulopathy.</description><identifier>ISSN: 0002-9440</identifier><identifier>EISSN: 1525-2191</identifier><identifier>DOI: 10.1016/S0002-9440(10)65264-9</identifier><identifier>PMID: 9916932</identifier><identifier>CODEN: AJPAA4</identifier><language>eng</language><publisher>Bethesda, MD: Elsevier Inc</publisher><subject>Animals ; Biological and medical sciences ; Complex syndromes ; Developmental Disabilities - metabolism ; Developmental Disabilities - pathology ; DNA-Binding Proteins - metabolism ; Female ; Female Urogenital Diseases - metabolism ; Female Urogenital Diseases - pathology ; Fetus ; Genitalia - abnormalities ; Genitalia - pathology ; Glomerular Mesangium - pathology ; Glomerulosclerosis, Focal Segmental - metabolism ; Glomerulosclerosis, Focal Segmental - pathology ; Humans ; Infant, Newborn ; Kidney Diseases - metabolism ; Kidney Diseases - pathology ; Kidney Glomerulus - pathology ; Male ; Male Urogenital Diseases ; Medical genetics ; Medical sciences ; PAX2 Transcription Factor ; Regular ; Sclerosis ; Syndrome ; Transcription Factors - metabolism ; WT1 Proteins</subject><ispartof>The American journal of pathology, 1999, Vol.154 (1), p.181-192</ispartof><rights>1999 American Society for Investigative Pathology</rights><rights>1999 INIST-CNRS</rights><rights>Copyright American Society for Investigative Pathology Jan 1999</rights><rights>Copyright © 1999, American Society for Investigative Pathology</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c667t-2503291d91313d5d8bae2f47b2258ee9062a797788c399a78d3216446967a2863</citedby><cites>FETCH-LOGICAL-c667t-2503291d91313d5d8bae2f47b2258ee9062a797788c399a78d3216446967a2863</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1853439/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0002-9440(10)65264-9$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,314,727,780,784,885,3550,4024,27923,27924,27925,45995,53791,53793</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1641305$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9916932$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Yang, Youxin</creatorcontrib><creatorcontrib>Jeanpierre, Cécile</creatorcontrib><creatorcontrib>Dressler, Gregory R.</creatorcontrib><creatorcontrib>Lacoste, Mireille</creatorcontrib><creatorcontrib>Niaudet, Patrick</creatorcontrib><creatorcontrib>Gubler, Marie-Claire</creatorcontrib><title>WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis</title><title>The American journal of pathology</title><addtitle>Am J Pathol</addtitle><description>Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is caused by dominant negative point mutations in the
WT1 gene that encodes a tumor suppressor transcription factor normally expressed in podocytes. Mutations usually affect the zinc fingers of the WT1 protein. The basic defect is unknown in most cases of isolated diffuse mesangial sclerosis, a disease characterized by the same glomerular changes as in Denys-Drash syndrome but possibly transmitted as an autosomal recessive trait. Here we show that the distribution of WT1 is abnormal in most patients with Denys-Drash syndrome: WT1 nuclear staining of podocytes is decreased or absent. This finding is consistent with the decreased DNA binding capacity of the mutated protein. One target gene of WT1 is
PAX2, the expression of which is down-regulated in podocytes during early stages of nephrogenesis. We demonstrate that WT1 mislocalization is associated with abnormal podocyte expression of PAX2 protein and RNA. We suggest that persistent expression of PAX2 is likely to result from the loss of WT1 dependent transcriptional repression and may participate in the pathological mechanisms leading to glomerular dysfunction. Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in
WT1 could also be operative in isolated diffuse mesangial sclerosis. Primary involvement of
PAX2 is an alternative hypothesis because persistent expression of PAX2 in transgenic mice is associated with the occurrence of early and severe glomerulopathy.</description><subject>Animals</subject><subject>Biological and medical sciences</subject><subject>Complex syndromes</subject><subject>Developmental Disabilities - metabolism</subject><subject>Developmental Disabilities - pathology</subject><subject>DNA-Binding Proteins - metabolism</subject><subject>Female</subject><subject>Female Urogenital Diseases - metabolism</subject><subject>Female Urogenital Diseases - pathology</subject><subject>Fetus</subject><subject>Genitalia - abnormalities</subject><subject>Genitalia - pathology</subject><subject>Glomerular Mesangium - pathology</subject><subject>Glomerulosclerosis, Focal Segmental - metabolism</subject><subject>Glomerulosclerosis, Focal Segmental - pathology</subject><subject>Humans</subject><subject>Infant, Newborn</subject><subject>Kidney Diseases - metabolism</subject><subject>Kidney Diseases - pathology</subject><subject>Kidney Glomerulus - pathology</subject><subject>Male</subject><subject>Male Urogenital Diseases</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>PAX2 Transcription Factor</subject><subject>Regular</subject><subject>Sclerosis</subject><subject>Syndrome</subject><subject>Transcription Factors - metabolism</subject><subject>WT1 Proteins</subject><issn>0002-9440</issn><issn>1525-2191</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1999</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqFkU9vEzEQxVcIVELhI1RaIYTgsODxrr32BVQ1BSoVUSlFcLMcezZxtLGDvSnk2-P8UQpcOFn2_ObNPL-iOAPyBgjwtxNCCK1k05BXQF5zRnlTyQfFCBhlFQUJD4vREXlcPElpka-8FuSkOJESuKzpqJh-u4VSe1venH-vaHkTbDCbAcvLX6uIKbngS-fLMfpNqsZRp3k52XgbwxJ3XVcp9HpAW45d160Tlp8xaT9zui8npscYkktPi0ed7hM-O5ynxdcPl7cXn6rrLx-vLs6vK8N5O1SUkZpKsBJqqC2zYqqRdk07pZQJREk41a1sWyFMLaVuha0p8KbhkreaCl6fFu_2uqv1dInWoB-i7tUquqWOGxW0U39XvJurWbhTIFjd1DILvDwIxPBjjWlQS5cM9r32GNZJcclYHkYy-PwfcBHW0WdzioKQQkIrMsT2kMm_kCJ2x02AqG2Capeg2sazfdolqLZbnP1p49h1iCzXXxzqOhndd1F749K9OG-gJuzezNzN5j9dRJWWuu-zKCi9WAFrFGTnkMH3exBzNncOo0rGoTdoc5MZlA3uPxv_BkWvwuE</recordid><startdate>1999</startdate><enddate>1999</enddate><creator>Yang, Youxin</creator><creator>Jeanpierre, Cécile</creator><creator>Dressler, Gregory R.</creator><creator>Lacoste, Mireille</creator><creator>Niaudet, Patrick</creator><creator>Gubler, Marie-Claire</creator><general>Elsevier Inc</general><general>ASIP</general><general>American Society for Investigative Pathology</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>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8C1</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>1999</creationdate><title>WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis</title><author>Yang, Youxin ; Jeanpierre, Cécile ; Dressler, Gregory R. ; Lacoste, Mireille ; Niaudet, Patrick ; Gubler, Marie-Claire</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c667t-2503291d91313d5d8bae2f47b2258ee9062a797788c399a78d3216446967a2863</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1999</creationdate><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>Complex syndromes</topic><topic>Developmental Disabilities - metabolism</topic><topic>Developmental Disabilities - pathology</topic><topic>DNA-Binding Proteins - metabolism</topic><topic>Female</topic><topic>Female Urogenital Diseases - metabolism</topic><topic>Female Urogenital Diseases - pathology</topic><topic>Fetus</topic><topic>Genitalia - abnormalities</topic><topic>Genitalia - pathology</topic><topic>Glomerular Mesangium - pathology</topic><topic>Glomerulosclerosis, Focal Segmental - metabolism</topic><topic>Glomerulosclerosis, Focal Segmental - pathology</topic><topic>Humans</topic><topic>Infant, Newborn</topic><topic>Kidney Diseases - metabolism</topic><topic>Kidney Diseases - pathology</topic><topic>Kidney Glomerulus - pathology</topic><topic>Male</topic><topic>Male Urogenital Diseases</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>PAX2 Transcription Factor</topic><topic>Regular</topic><topic>Sclerosis</topic><topic>Syndrome</topic><topic>Transcription Factors - metabolism</topic><topic>WT1 Proteins</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Yang, Youxin</creatorcontrib><creatorcontrib>Jeanpierre, Cécile</creatorcontrib><creatorcontrib>Dressler, Gregory R.</creatorcontrib><creatorcontrib>Lacoste, Mireille</creatorcontrib><creatorcontrib>Niaudet, Patrick</creatorcontrib><creatorcontrib>Gubler, Marie-Claire</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>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Science Database (Alumni Edition)</collection><collection>STEM Database</collection><collection>Public Health Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>The American journal of pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Yang, Youxin</au><au>Jeanpierre, Cécile</au><au>Dressler, Gregory R.</au><au>Lacoste, Mireille</au><au>Niaudet, Patrick</au><au>Gubler, Marie-Claire</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis</atitle><jtitle>The American journal of pathology</jtitle><addtitle>Am J Pathol</addtitle><date>1999</date><risdate>1999</risdate><volume>154</volume><issue>1</issue><spage>181</spage><epage>192</epage><pages>181-192</pages><issn>0002-9440</issn><eissn>1525-2191</eissn><coden>AJPAA4</coden><abstract>Denys-Drash syndrome is a rare disorder of urogenital development characterized by the association of early onset glomerulopathy caused by diffuse mesangial sclerosis, gonadal dysgenesis leading to pseudohermaphroditism in males, and a high risk of developing Wilms' tumor. The syndrome is caused by dominant negative point mutations in the
WT1 gene that encodes a tumor suppressor transcription factor normally expressed in podocytes. Mutations usually affect the zinc fingers of the WT1 protein. The basic defect is unknown in most cases of isolated diffuse mesangial sclerosis, a disease characterized by the same glomerular changes as in Denys-Drash syndrome but possibly transmitted as an autosomal recessive trait. Here we show that the distribution of WT1 is abnormal in most patients with Denys-Drash syndrome: WT1 nuclear staining of podocytes is decreased or absent. This finding is consistent with the decreased DNA binding capacity of the mutated protein. One target gene of WT1 is
PAX2, the expression of which is down-regulated in podocytes during early stages of nephrogenesis. We demonstrate that WT1 mislocalization is associated with abnormal podocyte expression of PAX2 protein and RNA. We suggest that persistent expression of PAX2 is likely to result from the loss of WT1 dependent transcriptional repression and may participate in the pathological mechanisms leading to glomerular dysfunction. Abnormal distribution of WT1 and PAX2 was also observed in isolated diffuse mesangial sclerosis suggesting that a defect in
WT1 could also be operative in isolated diffuse mesangial sclerosis. Primary involvement of
PAX2 is an alternative hypothesis because persistent expression of PAX2 in transgenic mice is associated with the occurrence of early and severe glomerulopathy.</abstract><cop>Bethesda, MD</cop><pub>Elsevier Inc</pub><pmid>9916932</pmid><doi>10.1016/S0002-9440(10)65264-9</doi><tpages>12</tpages><oa>free_for_read</oa></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0002-9440 |
ispartof | The American journal of pathology, 1999, Vol.154 (1), p.181-192 |
issn | 0002-9440 1525-2191 |
language | eng |
recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1853439 |
source | MEDLINE; Access via ScienceDirect (Elsevier); EZB-FREE-00999 freely available EZB journals; PubMed Central |
subjects | Animals Biological and medical sciences Complex syndromes Developmental Disabilities - metabolism Developmental Disabilities - pathology DNA-Binding Proteins - metabolism Female Female Urogenital Diseases - metabolism Female Urogenital Diseases - pathology Fetus Genitalia - abnormalities Genitalia - pathology Glomerular Mesangium - pathology Glomerulosclerosis, Focal Segmental - metabolism Glomerulosclerosis, Focal Segmental - pathology Humans Infant, Newborn Kidney Diseases - metabolism Kidney Diseases - pathology Kidney Glomerulus - pathology Male Male Urogenital Diseases Medical genetics Medical sciences PAX2 Transcription Factor Regular Sclerosis Syndrome Transcription Factors - metabolism WT1 Proteins |
title | WT1 and PAX-2 Podocyte Expression in Denys-Drash Syndrome and Isolated Diffuse Mesangial Sclerosis |
url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-25T03%3A11%3A01IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=WT1%20and%20PAX-2%20Podocyte%20Expression%20in%20Denys-Drash%20Syndrome%20and%20Isolated%20Diffuse%20Mesangial%20Sclerosis&rft.jtitle=The%20American%20journal%20of%20pathology&rft.au=Yang,%20Youxin&rft.date=1999&rft.volume=154&rft.issue=1&rft.spage=181&rft.epage=192&rft.pages=181-192&rft.issn=0002-9440&rft.eissn=1525-2191&rft.coden=AJPAA4&rft_id=info:doi/10.1016/S0002-9440(10)65264-9&rft_dat=%3Cproquest_pubme%3E69559670%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=218989178&rft_id=info:pmid/9916932&rft_els_id=S0002944010652649&rfr_iscdi=true |