Expression of Fraser syndrome genes in normal and polycystic murine kidneys
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- 作者：Larissa Kerecuk (12)
David A. Long (1)
Zahabia Ali (1)
Corina Anders (3)
Maria Kolatsi-Joannou (1)
Peter J. Scambler (1)
Adrian S. Woolf (34) adrian.woolf@manchester.ac.uk - 关键词：Basement membrane – ; Cyst – ; Development – ; Fras1 – ; Frem2 – ; LacZ – ; Reporter gene
- 刊名：Pediatric Nephrology
- 出版年：2012
- 出版时间：June 2012
- 年：2012
- 卷：27
- 期：6
- 页码：991-998
- 全文大小：678.8 KB
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- 刊物类别：Medicine
- 刊物主题：Medicine & Public Health
Pediatrics - 出版者：Springer Berlin / Heidelberg
- ISSN：1432-198X
文摘
Background Fraser syndrome (FS) features renal agenesis and cystic kidneys. Mutations of FRAS1 (Fraser syndrome 1) and FREM2 (FRAS1-related extracellular matrix protein 2) cause FS. They code for basement membrane proteins expressed in metanephric epithelia where they mediate epithelial/mesenchymal signalling. Little is known about whether and where these molecules are expressed in more mature kidneys.