The extracellular matrix and ciliary signaling
Open accessAbstract
The primary cilium protrudes like an antenna from the cell surface, sensing mechanical and chemical cues provided in the cellular environment. In some tissue types, ciliary orientation to lumens allows response to fluid flow; in others, such as bone, ciliary protrusion into the extracellular matrix allows response to compression forces. The ciliary membrane contains receptors for Hedgehog, Wnt, Notch, and other potent growth factors, and in some instances also harbors integrin and cadherin family members, allowing receipt of a robust range of signals. A growing list of ciliopathies, arising from deficient formation or function of cilia, includes both developmental defects and chronic, progressive disorders such as polycystic kidney disease (PKD); changes in ciliary function have been proposed to support cancer progression. Recent findings have revealed extensive signaling dialog between cilia and extracellular matrix (ECM), with defects in cilia associated with fibrosis in multiple contexts. Further, a growing number of proteins have been determined to possess multiple roles in control of cilia and focal adhesion interactions with the ECM, further coordinating functionality. We summarize and discuss these recent findings.
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The mechanisms that control the balanced output of ciliary signaling described in this Review might also apply to other pathways such as Hippo and Notch signaling pathways or those regulated by receptors of extracellular matrix molecules, which also have been linked to primary cilia in various cell types and tissues.
“The mechanisms that control the balanced output of ciliary signalling described in this Review might also apply to other pathways such as Hippo and Notch signalling pathways or those regulated by receptors of extracellular matrix molecules, which also have been linked to primary cilia in various cell types and tissues 23, 291, 292.”
From Cellular signalling by primary cilia in development, organ function and disease (2019) · cites this paper as [291]
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Self-citationNek2 activation of Kif24 ensures cilium disassembly during the cell cycle
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Primary cilia defects causing mitral valve prolapse
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ALMS1 and Alström syndrome: a recessive form of metabolic, neurosensory and cardiac deficits
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Endocytic Control of Cellular Signaling at the Primary Cilium
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The cilium as a force sensor−myth versus reality
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The intraflagellar transport machinery in ciliary signaling
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Targeting sonic hedgehog signaling in neurological disorders
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The Membrane Proteome of Sensory Cilia to the Depth of Olfactory Receptors
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Myristoylated CIL-7 regulates ciliary extracellular vesicle biogenesis
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Chlamydomonas PKD2 organizes mastigonemes, hair-like glycoprotein polymers on cilia
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Mitral Valve Prolapse Induces Regionalized Myocardial Fibrosis
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Mutations of ADAMTS9 Cause Nephronophthisis-Related Ciliopathy
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Ciliary IFT80 is essential for intervertebral disc development and maintenance
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RAB19 Directs Cortical Remodeling and Membrane Growth for Primary Ciliogenesis
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Ganetespib limits ciliation and cystogenesis in autosomal‐dominant polycystic kidney disease (ADPKD)
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Permeability barriers for generating a unique ciliary protein and lipid composition
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Regulation of the Extracellular Matrix by Ciliary Machinery
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Whole-genome screen identifies diverse pathways that negatively regulate ciliogenesis
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Cilia, Centrosomes and Skeletal Muscle
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Unexpected Activities in Regulating Ciliation Contribute to Off-target Effects of Targeted Drugs
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Self-citationIncreased Primary Cilia in Idiopathic Pulmonary Fibrosis.
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Primary Cilia and Dendritic Spines: Different but Similar Signaling Compartments
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Integrin α8 and Pcdh15 act as a complex to regulate cilia biogenesis in sensory cells
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Primary Ciliary Signaling in the Skin—Contribution to Wound Healing and Scarring
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Patterns of Ciliation and Ciliary Signaling in Cancer
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Simultaneous Measurement of Smoothened Entry Into and Exit From the Primary Cilium
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- Authors
- Seeger-Nukpezah, T., & Golemis, E. A.
- Affiliations
- Fox Chase Cancer Center (US)
- Corresponding author
- Golemis, Erica A
- ORCID
- Golemis, Erica A
- Venue
- Current Opinion in Cell Biology
- Publisher
- Elsevier BV
- Published
- 2012-10
- Volume
- 24
- Issue
- 5
- Pages
- 652-661
- ISSN
- 0955-0674 (ISSN-L)
- PMC
- PMC3479303
- OpenAlex
- W2150364654
- Cited by
- 117
- References
- 115
- Citations by year
- 2026: 5 · 2025: 6 · 2024: 7 · 2023: 5 · 2022: 11 · 2021: 9 · 2020: 12 · 2019: 6 · 2018: 8 · 2017: 7 · 2016: 12 · 2015: 12 · 2014: 12 · 2013: 5
- FWCI
- 1.13
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Current Opinion in Cell Biology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Cancer Institute (R01CA063366)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular Mechanics and Interactions
- Keywords
- primary cilia, extracellular matrix, ciliary signaling, ciliopathies, Hedgehog signaling, Wnt signaling, Notch signaling, integrins, cadherins, polycystic kidney disease, fibrosis, focal adhesions, mechanosensing, tumor progression
- MeSH terms
- Animals, Cell Adhesion, Cilia, Extracellular Matrix, Humans, Polycystic Kidney Diseases, Membrane Proteins, Signal Transduction
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