Cilia in cystic kidney and other diseases
| Autor: | Abigail O. Smith, Miriam Schmidts, Paurav B. Desai, Gregory J. Pazour, Lynne M. Quarmby |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Liver Cirrhosis TRPP Cation Channels Cystic Fibrosis Fibrocystin Receptors Cell Surface Nephron Biology Article 03 medical and health sciences 0302 clinical medicine Intraflagellar transport medicine Polycystic kidney disease Animals Humans Cilia Cystic kidney Kidney urogenital system Cilium Cell Biology Kidney Diseases Cystic medicine.disease 3. Good health Cell biology 030104 developmental biology medicine.anatomical_structure Tubule 030220 oncology & carcinogenesis biology.protein |
| Zdroj: | Cell Signal Cellular Signalling |
| ISSN: | 1873-3913 |
| Popis: | Epithelial cells lining the ducts and tubules of the kidney nephron and collecting duct have a single non-motile cilium projecting from their surface into the lumen of the tubule. These organelles were long considered vestigial remnants left as a result of evolution from a ciliated ancestor, but we now recognize them as critical sensory antennae. In the kidney, the polycystins and fibrocystin, products of the major human polycystic kidney disease genes, localize to this organelle. The polycystins and fibrocystin, through an unknown mechanism, monitor the diameter of the kidney tubules and regulate the proliferation and differentiation of the cells lining the tubule. When the polycystins, fibrocystin or cilia themselves are defective, the cell perceives this as a pro-proliferative signal, which leads to tubule dilation and cystic disease. In addition to critical roles in preventing cyst formation in the kidney, cilia are also important in cystic and fibrotic diseases of the liver and pancreas, and ciliary defects lead to a variety of developmental abnormalities that cause structural birth defects in most organs. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect