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| Cilia and centrosomes: A unifying pathogenic concept for cystic kidney disease? |
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| Author(s): Hildebrandt F, Otto E |
| Source: NATURE REVIEWS GENETICS Volume: 6 Issue: 12 Pages: 928-940 Published: DEC 2005 |
| Times Cited: 94 References: 145 |
| Abstract: Cystic kidney diseases are among the most frequent lethal genetic diseases. Positional cloning of novel cystic kidney disease genes revealed that their products (cystoproteins) are expressed in sensory organelles called primary cilia, in basal bodies or in centrosomes. Primary cilia link mechanosensory, visual, osmotic, gustatory and other stimuli to mechanisms of cell-cycle control and epithelial cell polarity. The ciliary expression of cystoproteins explains why many other organs might be also affected in patients with cystic kidney disease. Protein-protein interactions among cystoproteins, and their strong evolutionary conservation, provide a basis for a multidisciplinary approach to unravelling the novel signalling mechanisms that are involved in this disease group. |
| Document Type: Review |
| Language: English |
| Reprint Address: Hildebrandt, F (reprint author), Univ Michigan, Dept Pediat, 8220C MSRB 3,1150 W Med Ctr Dr, Ann Arbor, MI 48109 USA |
Addresses:
1. Univ Michigan, Dept Pediat, Ann Arbor, MI 48109 USA
2. Univ Michigan, Dept Human Genet, Ann Arbor, MI 48109 USA |
| Publisher: NATURE PUBLISHING GROUP, MACMILLAN BUILDING, 4 CRINAN ST, LONDON N1 9XW, ENGLAND |
| Subject Category: Genetics & Heredity |
| IDS Number: 989FF |
| ISSN: 1471-0056 |
| DOI: 10.1038/nrg1727 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |