Home > Publications Database > A cAMP signalosome in primary cilia drives gene expression and kidney cyst formation > print

001     164661
005     20240313115347.0
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037 _ _ |a DZNE-2022-01191
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Hansen, Jan N
|b 0
245 _ _ |a A cAMP signalosome in primary cilia drives gene expression and kidney cyst formation
260 _ _ |a Hoboken, NJ [u.a.]
|c 2022
|b Wiley
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500 _ _ |a (CC BY-NC-ND)
520 _ _ |a The primary cilium constitutes an organelle that orchestrates signal transduction independently from the cell body. Dysregulation of this intricate molecular architecture leads to severe human diseases, commonly referred to as ciliopathies. However, the molecular underpinnings how ciliary signaling orchestrates a specific cellular output remain elusive. By combining spatially resolved optogenetics with RNA sequencing and imaging, we reveal a novel cAMP signalosome that is functionally distinct from the cytoplasm. We identify the genes and pathways targeted by the ciliary cAMP signalosome and shed light on the underlying mechanisms and downstream signaling. We reveal that chronic stimulation of the ciliary cAMP signalosome transforms kidney epithelia from tubules into cysts. Counteracting this chronic cAMP elevation in the cilium by small molecules targeting activation of phosphodiesterase-4 long isoforms inhibits cyst growth. Thereby, we identify a novel concept of how the primary cilium controls cellular functions and maintains tissue integrity in a specific and spatially distinct manner and reveal novel molecular components that might be involved in the development of one of the most common genetic diseases, polycystic kidney disease.
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650 _ 7 |a CREB
|2 Other
650 _ 7 |a PKD
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650 _ 7 |a cAMP
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650 _ 7 |a optogenetics
|2 Other
650 _ 7 |a primary cilia
|2 Other
650 _ 2 |a Cilia: metabolism
|2 MeSH
650 _ 2 |a Cysts: metabolism
|2 MeSH
650 _ 2 |a Gene Expression
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Kidney
|2 MeSH
650 _ 2 |a Polycystic Kidney Diseases: genetics
|2 MeSH
650 _ 2 |a Polycystic Kidney Diseases: metabolism
|2 MeSH
700 1 _ |a Kaiser, Fabian
|b 1
700 1 _ |a Leyendecker, Philipp
|b 2
700 1 _ |a Stüven, Birthe
|b 3
700 1 _ |a Krause, Jens-Henning
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700 1 _ |a Derakhshandeh, Fatemeh
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700 1 _ |a Irfan, Jaazba
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700 1 _ |a Sroka, Tommy J
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700 1 _ |a Preval, Kenley M
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700 1 _ |a Desai, Paurav B
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700 1 _ |a Kraut, Michael
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700 1 _ |a Theis, Heidi
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700 1 _ |a Drews, Anna-Dorothee
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700 1 _ |a de Domenico, Elena
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700 1 _ |a Händler, Kristian
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700 1 _ |a Pazour, Gregory J
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700 1 _ |a Henderson, David J P
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700 1 _ |a Mick, David U
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700 1 _ |a Wachten, Dagmar
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773 _ _ |a 10.15252/embr.202154315
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