A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome
The Wilms tumor-suppressor gene, WT1, plays a key role in urogenital development, and WT1 dysfunction is implicated in both neoplastic (Wilms tumor, mesothelioma, leukemias, and breast cancer) and nonneoplastic (glomerulosclerosis) disease. The analysis of diseases linked specifically with WT1 mutat...
Gespeichert in:
| Veröffentlicht in: | Proceedings of the National Academy of Sciences - PNAS 1999-03, Vol.96 (6), p.2931-2936 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 2936 |
|---|---|
| container_issue | 6 |
| container_start_page | 2931 |
| container_title | Proceedings of the National Academy of Sciences - PNAS |
| container_volume | 96 |
| creator | Patek, Charles E. Little, Melissa H. Fleming, Stewart Miles, Colin Charlieu, Jean-Paul Clarke, Alan R. Miyagawa, Kiyoshi Christie, Sheila Doig, Jennifer Harrison, David J. Porteous, David J. Brookes, Anthony J. Hooper, Martin L. Hastie, Nicholas D. |
| description | The Wilms tumor-suppressor gene, WT1, plays a key role in urogenital development, and WT1 dysfunction is implicated in both neoplastic (Wilms tumor, mesothelioma, leukemias, and breast cancer) and nonneoplastic (glomerulosclerosis) disease. The analysis of diseases linked specifically with WT1 mutations, such as Denys-Drash syndrome (DDS), can provide valuable insight concerning the role of WT1 in development and disease. DDS is a rare childhood disease characterized by a nephropathy involving mesangial sclerosis, XY pseudohermaphroditism, and/or Wilms tumor (WT). DDS patients are constitutionally heterozygous for exonic point mutations in WT1, which include mutations predicted to truncate the protein within the C-terminal zinc finger (ZF) region. We report that heterozygosity for a targeted murine Wt1 allele, Wt1tmT396, which truncates ZF3 at codon 396, induces mesangial sclerosis characteristic of DDS in adult heterozygous and chimeric mice. Male genital defects also were evident and there was a single case of Wilms tumor in which the transcript of the nontargeted allele showed an exon 9 skipping event, implying a causal link between Wt1 dysfunction and Wilms tumorigenesis in mice. However, the mutant WT1tmT396protein accounted for only 5% of WT1 in both heterozygous embryonic stem cells and the WT. This has implications regarding the mechanism by which the mutant allele exerts its effect. |
| doi_str_mv | 10.1073/pnas.96.6.2931 |
| format | Article |
| fullrecord | <record><control><sourceid>jstor_proqu</sourceid><recordid>TN_cdi_proquest_miscellaneous_17214853</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><jstor_id>47466</jstor_id><sourcerecordid>47466</sourcerecordid><originalsourceid>FETCH-LOGICAL-c582t-506985ea0b8e86e4852e5dc93c19263e2d01e733ca47bc0ddba9f8be68e2c9f13</originalsourceid><addsrcrecordid>eNqF0c1v0zAYBnALgVgZXDkggSwOuyX4I3FiaZeq2wBpCAk6IXGxHOdN6yq1i-2g9b8nUctUOMDJh_f3vLL9IPSSkpySir_bOR1zKXKRM8npIzSjRNJMFJI8RjNCWJXVBSvO0LMYN4QQWdbkKTqjhFSVoMUMDXP83TqDb6xbQcDLMDijk_UO-w5_GoJ1gL8tKf4CcehTxNbhtAa8WOugTYJgY7IG3wW_AmeT7vG8cT5sdW-ThTgtuQK3j9lV0HGNv-5dG_wWnqMnne4jvDie5-ju5nq5-JDdfn7_cTG_zUxZs5SVRMi6BE2aGmoBRV0yKFsjuaGSCQ6sJRQqzo0uqsaQtm207OoGRA3MyI7yc3R52Lsbmi20BlwKule7YLc67JXXVv05cXatVv6nomVdsTF-cYwH_2OAmNTWRgN9rx34ISohBZMlLf8LacXoeHs-wrd_wY0fghv_QDFC-YiqCeUHZIKPMUD3cGFK1NS6mlpXUiihptbHwJvTZ57wQ80jeH0EU_D3-HTBxb_mqhv6PsF9GuGrA9zE5MODLKpCCP4LrGbK6Q</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>201314873</pqid></control><display><type>article</type><title>A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome</title><source>MEDLINE</source><source>JSTOR Archive Collection A-Z Listing</source><source>PubMed Central</source><source>Alma/SFX Local Collection</source><source>Free Full-Text Journals in Chemistry</source><creator>Patek, Charles E. ; Little, Melissa H. ; Fleming, Stewart ; Miles, Colin ; Charlieu, Jean-Paul ; Clarke, Alan R. ; Miyagawa, Kiyoshi ; Christie, Sheila ; Doig, Jennifer ; Harrison, David J. ; Porteous, David J. ; Brookes, Anthony J. ; Hooper, Martin L. ; Hastie, Nicholas D.</creator><creatorcontrib>Patek, Charles E. ; Little, Melissa H. ; Fleming, Stewart ; Miles, Colin ; Charlieu, Jean-Paul ; Clarke, Alan R. ; Miyagawa, Kiyoshi ; Christie, Sheila ; Doig, Jennifer ; Harrison, David J. ; Porteous, David J. ; Brookes, Anthony J. ; Hooper, Martin L. ; Hastie, Nicholas D.</creatorcontrib><description>The Wilms tumor-suppressor gene, WT1, plays a key role in urogenital development, and WT1 dysfunction is implicated in both neoplastic (Wilms tumor, mesothelioma, leukemias, and breast cancer) and nonneoplastic (glomerulosclerosis) disease. The analysis of diseases linked specifically with WT1 mutations, such as Denys-Drash syndrome (DDS), can provide valuable insight concerning the role of WT1 in development and disease. DDS is a rare childhood disease characterized by a nephropathy involving mesangial sclerosis, XY pseudohermaphroditism, and/or Wilms tumor (WT). DDS patients are constitutionally heterozygous for exonic point mutations in WT1, which include mutations predicted to truncate the protein within the C-terminal zinc finger (ZF) region. We report that heterozygosity for a targeted murine Wt1 allele, Wt1tmT396, which truncates ZF3 at codon 396, induces mesangial sclerosis characteristic of DDS in adult heterozygous and chimeric mice. Male genital defects also were evident and there was a single case of Wilms tumor in which the transcript of the nontargeted allele showed an exon 9 skipping event, implying a causal link between Wt1 dysfunction and Wilms tumorigenesis in mice. However, the mutant WT1tmT396protein accounted for only 5% of WT1 in both heterozygous embryonic stem cells and the WT. This has implications regarding the mechanism by which the mutant allele exerts its effect.</description><identifier>ISSN: 0027-8424</identifier><identifier>EISSN: 1091-6490</identifier><identifier>DOI: 10.1073/pnas.96.6.2931</identifier><identifier>PMID: 10077614</identifier><language>eng</language><publisher>United States: National Academy of Sciences of the United States of America</publisher><subject>Animals ; Base Sequence ; Biological Sciences ; Cell lines ; Chimeras ; Disease ; DNA-Binding Proteins - genetics ; Exons ; Genes ; Genes, Wilms Tumor ; Genetic Linkage ; Genetic mutation ; Genetics ; Genitalia, Male - abnormalities ; Heterozygotes ; Humans ; Kidney Diseases - genetics ; Kidneys ; Male ; Mice ; Molecular Sequence Data ; Mutation ; Nephrons ; Sertoli cells ; Sex chromosomes ; Syndrome ; Transcription Factors - genetics ; Tumors ; Wilms tumors ; WT1 Proteins ; Zinc ; Zinc Fingers - genetics</subject><ispartof>Proceedings of the National Academy of Sciences - PNAS, 1999-03, Vol.96 (6), p.2931-2936</ispartof><rights>Copyright 1993-1999 The National Academy of Sciences of the United States of America</rights><rights>Copyright National Academy of Sciences Mar 16, 1999</rights><rights>Copyright © 1999, The National Academy of Sciences 1999</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c582t-506985ea0b8e86e4852e5dc93c19263e2d01e733ca47bc0ddba9f8be68e2c9f13</citedby><cites>FETCH-LOGICAL-c582t-506985ea0b8e86e4852e5dc93c19263e2d01e733ca47bc0ddba9f8be68e2c9f13</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Uhttp://www.pnas.org/content/96/6.cover.gif</thumbnail><linktopdf>$$Uhttps://www.jstor.org/stable/pdf/47466$$EPDF$$P50$$Gjstor$$H</linktopdf><linktohtml>$$Uhttps://www.jstor.org/stable/47466$$EHTML$$P50$$Gjstor$$H</linktohtml><link.rule.ids>230,314,727,780,784,803,885,27924,27925,53791,53793,58017,58250</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10077614$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Patek, Charles E.</creatorcontrib><creatorcontrib>Little, Melissa H.</creatorcontrib><creatorcontrib>Fleming, Stewart</creatorcontrib><creatorcontrib>Miles, Colin</creatorcontrib><creatorcontrib>Charlieu, Jean-Paul</creatorcontrib><creatorcontrib>Clarke, Alan R.</creatorcontrib><creatorcontrib>Miyagawa, Kiyoshi</creatorcontrib><creatorcontrib>Christie, Sheila</creatorcontrib><creatorcontrib>Doig, Jennifer</creatorcontrib><creatorcontrib>Harrison, David J.</creatorcontrib><creatorcontrib>Porteous, David J.</creatorcontrib><creatorcontrib>Brookes, Anthony J.</creatorcontrib><creatorcontrib>Hooper, Martin L.</creatorcontrib><creatorcontrib>Hastie, Nicholas D.</creatorcontrib><title>A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome</title><title>Proceedings of the National Academy of Sciences - PNAS</title><addtitle>Proc Natl Acad Sci U S A</addtitle><description>The Wilms tumor-suppressor gene, WT1, plays a key role in urogenital development, and WT1 dysfunction is implicated in both neoplastic (Wilms tumor, mesothelioma, leukemias, and breast cancer) and nonneoplastic (glomerulosclerosis) disease. The analysis of diseases linked specifically with WT1 mutations, such as Denys-Drash syndrome (DDS), can provide valuable insight concerning the role of WT1 in development and disease. DDS is a rare childhood disease characterized by a nephropathy involving mesangial sclerosis, XY pseudohermaphroditism, and/or Wilms tumor (WT). DDS patients are constitutionally heterozygous for exonic point mutations in WT1, which include mutations predicted to truncate the protein within the C-terminal zinc finger (ZF) region. We report that heterozygosity for a targeted murine Wt1 allele, Wt1tmT396, which truncates ZF3 at codon 396, induces mesangial sclerosis characteristic of DDS in adult heterozygous and chimeric mice. Male genital defects also were evident and there was a single case of Wilms tumor in which the transcript of the nontargeted allele showed an exon 9 skipping event, implying a causal link between Wt1 dysfunction and Wilms tumorigenesis in mice. However, the mutant WT1tmT396protein accounted for only 5% of WT1 in both heterozygous embryonic stem cells and the WT. This has implications regarding the mechanism by which the mutant allele exerts its effect.</description><subject>Animals</subject><subject>Base Sequence</subject><subject>Biological Sciences</subject><subject>Cell lines</subject><subject>Chimeras</subject><subject>Disease</subject><subject>DNA-Binding Proteins - genetics</subject><subject>Exons</subject><subject>Genes</subject><subject>Genes, Wilms Tumor</subject><subject>Genetic Linkage</subject><subject>Genetic mutation</subject><subject>Genetics</subject><subject>Genitalia, Male - abnormalities</subject><subject>Heterozygotes</subject><subject>Humans</subject><subject>Kidney Diseases - genetics</subject><subject>Kidneys</subject><subject>Male</subject><subject>Mice</subject><subject>Molecular Sequence Data</subject><subject>Mutation</subject><subject>Nephrons</subject><subject>Sertoli cells</subject><subject>Sex chromosomes</subject><subject>Syndrome</subject><subject>Transcription Factors - genetics</subject><subject>Tumors</subject><subject>Wilms tumors</subject><subject>WT1 Proteins</subject><subject>Zinc</subject><subject>Zinc Fingers - genetics</subject><issn>0027-8424</issn><issn>1091-6490</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1999</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqF0c1v0zAYBnALgVgZXDkggSwOuyX4I3FiaZeq2wBpCAk6IXGxHOdN6yq1i-2g9b8nUctUOMDJh_f3vLL9IPSSkpySir_bOR1zKXKRM8npIzSjRNJMFJI8RjNCWJXVBSvO0LMYN4QQWdbkKTqjhFSVoMUMDXP83TqDb6xbQcDLMDijk_UO-w5_GoJ1gL8tKf4CcehTxNbhtAa8WOugTYJgY7IG3wW_AmeT7vG8cT5sdW-ThTgtuQK3j9lV0HGNv-5dG_wWnqMnne4jvDie5-ju5nq5-JDdfn7_cTG_zUxZs5SVRMi6BE2aGmoBRV0yKFsjuaGSCQ6sJRQqzo0uqsaQtm207OoGRA3MyI7yc3R52Lsbmi20BlwKule7YLc67JXXVv05cXatVv6nomVdsTF-cYwH_2OAmNTWRgN9rx34ISohBZMlLf8LacXoeHs-wrd_wY0fghv_QDFC-YiqCeUHZIKPMUD3cGFK1NS6mlpXUiihptbHwJvTZ57wQ80jeH0EU_D3-HTBxb_mqhv6PsF9GuGrA9zE5MODLKpCCP4LrGbK6Q</recordid><startdate>19990316</startdate><enddate>19990316</enddate><creator>Patek, Charles E.</creator><creator>Little, Melissa H.</creator><creator>Fleming, Stewart</creator><creator>Miles, Colin</creator><creator>Charlieu, Jean-Paul</creator><creator>Clarke, Alan R.</creator><creator>Miyagawa, Kiyoshi</creator><creator>Christie, Sheila</creator><creator>Doig, Jennifer</creator><creator>Harrison, David J.</creator><creator>Porteous, David J.</creator><creator>Brookes, Anthony J.</creator><creator>Hooper, Martin L.</creator><creator>Hastie, Nicholas D.</creator><general>National Academy of Sciences of the United States of America</general><general>National Acad Sciences</general><general>National Academy of Sciences</general><general>The National Academy of Sciences</general><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>7QG</scope><scope>7QL</scope><scope>7QP</scope><scope>7QR</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TK</scope><scope>7TM</scope><scope>7TO</scope><scope>7U9</scope><scope>8FD</scope><scope>C1K</scope><scope>FR3</scope><scope>H94</scope><scope>M7N</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>19990316</creationdate><title>A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome</title><author>Patek, Charles E. ; Little, Melissa H. ; Fleming, Stewart ; Miles, Colin ; Charlieu, Jean-Paul ; Clarke, Alan R. ; Miyagawa, Kiyoshi ; Christie, Sheila ; Doig, Jennifer ; Harrison, David J. ; Porteous, David J. ; Brookes, Anthony J. ; Hooper, Martin L. ; Hastie, Nicholas D.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c582t-506985ea0b8e86e4852e5dc93c19263e2d01e733ca47bc0ddba9f8be68e2c9f13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1999</creationdate><topic>Animals</topic><topic>Base Sequence</topic><topic>Biological Sciences</topic><topic>Cell lines</topic><topic>Chimeras</topic><topic>Disease</topic><topic>DNA-Binding Proteins - genetics</topic><topic>Exons</topic><topic>Genes</topic><topic>Genes, Wilms Tumor</topic><topic>Genetic Linkage</topic><topic>Genetic mutation</topic><topic>Genetics</topic><topic>Genitalia, Male - abnormalities</topic><topic>Heterozygotes</topic><topic>Humans</topic><topic>Kidney Diseases - genetics</topic><topic>Kidneys</topic><topic>Male</topic><topic>Mice</topic><topic>Molecular Sequence Data</topic><topic>Mutation</topic><topic>Nephrons</topic><topic>Sertoli cells</topic><topic>Sex chromosomes</topic><topic>Syndrome</topic><topic>Transcription Factors - genetics</topic><topic>Tumors</topic><topic>Wilms tumors</topic><topic>WT1 Proteins</topic><topic>Zinc</topic><topic>Zinc Fingers - genetics</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Patek, Charles E.</creatorcontrib><creatorcontrib>Little, Melissa H.</creatorcontrib><creatorcontrib>Fleming, Stewart</creatorcontrib><creatorcontrib>Miles, Colin</creatorcontrib><creatorcontrib>Charlieu, Jean-Paul</creatorcontrib><creatorcontrib>Clarke, Alan R.</creatorcontrib><creatorcontrib>Miyagawa, Kiyoshi</creatorcontrib><creatorcontrib>Christie, Sheila</creatorcontrib><creatorcontrib>Doig, Jennifer</creatorcontrib><creatorcontrib>Harrison, David J.</creatorcontrib><creatorcontrib>Porteous, David J.</creatorcontrib><creatorcontrib>Brookes, Anthony J.</creatorcontrib><creatorcontrib>Hooper, Martin L.</creatorcontrib><creatorcontrib>Hastie, Nicholas D.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Animal Behavior Abstracts</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Chemoreception Abstracts</collection><collection>Ecology Abstracts</collection><collection>Entomology Abstracts (Full archive)</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Technology Research Database</collection><collection>Environmental Sciences and Pollution Management</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Proceedings of the National Academy of Sciences - PNAS</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Patek, Charles E.</au><au>Little, Melissa H.</au><au>Fleming, Stewart</au><au>Miles, Colin</au><au>Charlieu, Jean-Paul</au><au>Clarke, Alan R.</au><au>Miyagawa, Kiyoshi</au><au>Christie, Sheila</au><au>Doig, Jennifer</au><au>Harrison, David J.</au><au>Porteous, David J.</au><au>Brookes, Anthony J.</au><au>Hooper, Martin L.</au><au>Hastie, Nicholas D.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome</atitle><jtitle>Proceedings of the National Academy of Sciences - PNAS</jtitle><addtitle>Proc Natl Acad Sci U S A</addtitle><date>1999-03-16</date><risdate>1999</risdate><volume>96</volume><issue>6</issue><spage>2931</spage><epage>2936</epage><pages>2931-2936</pages><issn>0027-8424</issn><eissn>1091-6490</eissn><abstract>The Wilms tumor-suppressor gene, WT1, plays a key role in urogenital development, and WT1 dysfunction is implicated in both neoplastic (Wilms tumor, mesothelioma, leukemias, and breast cancer) and nonneoplastic (glomerulosclerosis) disease. The analysis of diseases linked specifically with WT1 mutations, such as Denys-Drash syndrome (DDS), can provide valuable insight concerning the role of WT1 in development and disease. DDS is a rare childhood disease characterized by a nephropathy involving mesangial sclerosis, XY pseudohermaphroditism, and/or Wilms tumor (WT). DDS patients are constitutionally heterozygous for exonic point mutations in WT1, which include mutations predicted to truncate the protein within the C-terminal zinc finger (ZF) region. We report that heterozygosity for a targeted murine Wt1 allele, Wt1tmT396, which truncates ZF3 at codon 396, induces mesangial sclerosis characteristic of DDS in adult heterozygous and chimeric mice. Male genital defects also were evident and there was a single case of Wilms tumor in which the transcript of the nontargeted allele showed an exon 9 skipping event, implying a causal link between Wt1 dysfunction and Wilms tumorigenesis in mice. However, the mutant WT1tmT396protein accounted for only 5% of WT1 in both heterozygous embryonic stem cells and the WT. This has implications regarding the mechanism by which the mutant allele exerts its effect.</abstract><cop>United States</cop><pub>National Academy of Sciences of the United States of America</pub><pmid>10077614</pmid><doi>10.1073/pnas.96.6.2931</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0027-8424 |
| ispartof | Proceedings of the National Academy of Sciences - PNAS, 1999-03, Vol.96 (6), p.2931-2936 |
| issn | 0027-8424 1091-6490 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_17214853 |
| source | MEDLINE; JSTOR Archive Collection A-Z Listing; PubMed Central; Alma/SFX Local Collection; Free Full-Text Journals in Chemistry |
| subjects | Animals Base Sequence Biological Sciences Cell lines Chimeras Disease DNA-Binding Proteins - genetics Exons Genes Genes, Wilms Tumor Genetic Linkage Genetic mutation Genetics Genitalia, Male - abnormalities Heterozygotes Humans Kidney Diseases - genetics Kidneys Male Mice Molecular Sequence Data Mutation Nephrons Sertoli cells Sex chromosomes Syndrome Transcription Factors - genetics Tumors Wilms tumors WT1 Proteins Zinc Zinc Fingers - genetics |
| title | A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-05T07%3A05%3A48IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-jstor_proqu&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=A%20Zinc%20Finger%20Truncation%20of%20Murine%20WT1%20Results%20in%20the%20Characteristic%20Urogenital%20Abnormalities%20of%20Denys-Drash%20Syndrome&rft.jtitle=Proceedings%20of%20the%20National%20Academy%20of%20Sciences%20-%20PNAS&rft.au=Patek,%20Charles%20E.&rft.date=1999-03-16&rft.volume=96&rft.issue=6&rft.spage=2931&rft.epage=2936&rft.pages=2931-2936&rft.issn=0027-8424&rft.eissn=1091-6490&rft_id=info:doi/10.1073/pnas.96.6.2931&rft_dat=%3Cjstor_proqu%3E47466%3C/jstor_proqu%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=201314873&rft_id=info:pmid/10077614&rft_jstor_id=47466&rfr_iscdi=true |