Abstract | BACKGROUND: METHODS: RESULTS: Nascent nephrons transiently expressed Frem2 in both tubule and podocyte epithelia. Maturing and adult collecting ducts also expressed Frem2. Frem2 was expressed in cpk cystic epithelia although Frem2 haploinsufficiency did not significantly modify cystogenesis in vivo. Fras1 transcripts were significantly upregulated, and Frem3 downregulated, in polycystic kidneys versus the non- cystic kidneys of littermates. Fras1 was immunodetected in cpk, PCK and dexamethasone-induced cystepithelia. CONCLUSIONS: These descriptive results are consistent with the hypothesis that Fras family molecules play diverse roles in kidney epithelia. In future, this should be tested by conditional deletion of FS genes in nephron segments and collecting ducts.
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Authors | Larissa Kerecuk, David A Long, Zahabia Ali, Corina Anders, Maria Kolatsi-Joannou, Peter J Scambler, Adrian S Woolf |
Journal | Pediatric nephrology (Berlin, Germany)
(Pediatr Nephrol)
Vol. 27
Issue 6
Pg. 991-8
(Jun 2012)
ISSN: 1432-198X [Electronic] Germany |
PMID | 21993971
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Cys1 protein, mouse
- Extracellular Matrix Proteins
- Fras1 protein, mouse
- Frem2 protein, mouse
- Frem3 protein, mouse
- Membrane Proteins
- PKHD1 protein, rat
- Receptors, Cell Surface
- Dexamethasone
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Topics |
- Animals
- Dexamethasone
(pharmacology)
- Disease Models, Animal
- Embryo Culture Techniques
- Extracellular Matrix Proteins
(genetics, metabolism)
- Fraser Syndrome
(genetics, metabolism, pathology)
- Gene Expression Regulation
- Genes, Reporter
- Immunohistochemistry
- Lac Operon
- Membrane Proteins
(genetics, metabolism)
- Mice
- Mice, Inbred C57BL
- Mice, Transgenic
- Mutation
- Nephrons
(drug effects, embryology, metabolism, pathology)
- Polycystic Kidney, Autosomal Recessive
(genetics, metabolism, pathology)
- Rats
- Receptors, Cell Surface
(genetics)
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