Håkan Axelson
Research team manager
Simultaneous targeted activation of Notch1 and Vhl-disruption in the kidney proximal epithelial tubular cells in mice
Author
Summary, in English
Clear cell renal cell carcinoma (ccRCC) is the most common subtype of kidney cancer, representing approximately 75% of all renal neoplasms. ccRCC is known to be strongly associated with silencing of the von Hippel Lindau (VHL) tumor suppressor gene, yet VHL deficiency alone does not seem to be sufficient to drive the oncogenic transformation of normal renal epithelium and induce renal tumorigenesis. We, and others, have previously suggested that constitutive activation of the Notch signaling pathway, alongside with VHL loss, contribute to the oncogenic features of ccRCC. Here we report a prevailing hyperactivation of the Notch1 receptor in human ccRCC relative to the healthy counterpart. To explore the consequences of the elevated Notch1 signaling observed in ccRCC patient material, we made use of a conditional mouse model based on concurrent ectopic expression of constitutively active Notch1 (NICD1) and deletion of the Vhl gene. Histological examination of the kidneys of the conditional mice demonstrate the existence of nests of dysplastic cells with a clear cytoplasm as a consequence of lipid accumulation, thus displaying a one important hallmark of human ccRCC.
Department/s
- Kidney cancer research group
- Division of Translational Cancer Research
- Department of Translational Medicine
- Clinical pathology, Malmö
- BioCARE: Biomarkers in Cancer Medicine improving Health Care, Education and Innovation
- Division of Molecular Hematology (DMH)
Publishing year
2016-08-06
Language
English
Publication/Series
Scientific Reports
Volume
6
Document type
Journal article
Publisher
Nature Publishing Group
Topic
- Cancer and Oncology
- Medical Genetics
Status
Published
Research group
- Kidney cancer research group
- Clinical pathology, Malmö
ISBN/ISSN/Other
- ISSN: 2045-2322