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Article: A proteomic investigation of glomerular podocytes from a Denys-Drash Syndrome patient with a mutation in the Wilms tumour suppressor gene WT1

TitleA proteomic investigation of glomerular podocytes from a Denys-Drash Syndrome patient with a mutation in the Wilms tumour suppressor gene WT1
Authors
Issue Date2007
Citation
Proteomics, 2007, v. 7 n. 5, p. 804-815 How to Cite?
AbstractGlomerular podocytes are essential for blood filtration in the kidney underpinned by their unique cytoskeletal morphology. An increasing number of kidney diseases are being associated with key podocyte abnormalities. The Wilms tumour suppressor gene (WT1) encodes a zinc finger protein with a crucial role in normal kidney development; and in the adult, WT1 is required for normal podocyte function. Denys-Drash Syndrome (DDS) results from mutations affecting the zinc finger domain of WT1. The aim of this study was to undertake, for the first time, a proteomic analysis of cultured human podocytes; and to analyse the molecular changes in DDS podocytes. The morphology of DDS podocytes was highly irregular, reminiscent of a fibroblastic appearance. A reference 2-D gel was generated, and 75 proteins were identified of which 43% involved in cytoskeletal architecture. The DDS and wild-type proteomes were compared by 2-D DIGE. The level of 95.6% of proteins was unaltered; but 4.4% were altered more than two-fold. A sample of proteins involved in cytoskeletal architecture appeared to be misexpressed in DDS podocytes. Consistent with this finding, overall levels of filamentous actin also appeared reduced in DDS podocytes. We conclude that one of WT1 functions in podocytes is to regulate the expression of key components and regulators of the cytoskeleton. © 2007 Wiley-VCH Verlag GmbH & Co. KGaA.
Persistent Identifierhttp://hdl.handle.net/10722/195444
ISSN
2015 Impact Factor: 4.079
2015 SCImago Journal Rankings: 1.476
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorViney, RL-
dc.contributor.authorMorrison, AA-
dc.contributor.authorVan Den Heuver, LP-
dc.contributor.authorNi, L-
dc.contributor.authorMathieson, PW-
dc.contributor.authorSaleem, MA-
dc.contributor.authorLadomery, MR-
dc.date.accessioned2014-02-28T06:12:09Z-
dc.date.available2014-02-28T06:12:09Z-
dc.date.issued2007-
dc.identifier.citationProteomics, 2007, v. 7 n. 5, p. 804-815-
dc.identifier.issn1615-9853-
dc.identifier.urihttp://hdl.handle.net/10722/195444-
dc.description.abstractGlomerular podocytes are essential for blood filtration in the kidney underpinned by their unique cytoskeletal morphology. An increasing number of kidney diseases are being associated with key podocyte abnormalities. The Wilms tumour suppressor gene (WT1) encodes a zinc finger protein with a crucial role in normal kidney development; and in the adult, WT1 is required for normal podocyte function. Denys-Drash Syndrome (DDS) results from mutations affecting the zinc finger domain of WT1. The aim of this study was to undertake, for the first time, a proteomic analysis of cultured human podocytes; and to analyse the molecular changes in DDS podocytes. The morphology of DDS podocytes was highly irregular, reminiscent of a fibroblastic appearance. A reference 2-D gel was generated, and 75 proteins were identified of which 43% involved in cytoskeletal architecture. The DDS and wild-type proteomes were compared by 2-D DIGE. The level of 95.6% of proteins was unaltered; but 4.4% were altered more than two-fold. A sample of proteins involved in cytoskeletal architecture appeared to be misexpressed in DDS podocytes. Consistent with this finding, overall levels of filamentous actin also appeared reduced in DDS podocytes. We conclude that one of WT1 functions in podocytes is to regulate the expression of key components and regulators of the cytoskeleton. © 2007 Wiley-VCH Verlag GmbH & Co. KGaA.-
dc.languageeng-
dc.relation.ispartofProteomics-
dc.titleA proteomic investigation of glomerular podocytes from a Denys-Drash Syndrome patient with a mutation in the Wilms tumour suppressor gene WT1-
dc.typeArticle-
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1002/pmic.200600666-
dc.identifier.pmid17295355-
dc.identifier.scopuseid_2-s2.0-33947367874-
dc.identifier.volume7-
dc.identifier.issue5-
dc.identifier.spage804-
dc.identifier.epage815-
dc.identifier.isiWOS:000245158500015-

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