IFT27 Links the BBSome to IFT for Maintenance of the Ciliary Signaling Compartment
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Vertebrate hedgehog signaling is coordinated by the differential localization of the receptors patched-1 and Smoothened in the primary cilium. Cilia assembly is mediated by intraflagellar transport (IFT), and cilia defects disrupt hedgehog signaling, causing many structural birth defects. We generated Ift25 and Ift27 knockout mice and show that they have structural birth defects indicative of hedgehog signaling dysfunction. Surprisingly, ciliary assembly is not affected, but abnormal hedgehog signaling is observed in conjunction with ciliary accumulation of patched-1 and Smoothened. Similarly, Smoothened accumulates in cilia on cells mutated for BBSome components or the BBS binding protein/regulator Lztfl1. Interestingly, the BBSome and Lztfl1 accumulate to high levels in Ift27 mutant cilia. Because Lztfl1 mutant cells accumulate BBSome but not IFT27, it is likely that Lztfl1 functions downstream of IFT27 to couple the BBSome to the IFT particle for coordinated removal of patched-1 and Smoothened from cilia during hedgehog signaling.
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The IFT-B complex proteins IFT-81 and IFT-74 promote anterograde IFT of tubulin toward the ciliary tip during axoneme assembly.
“For instance, the IFT-B complex proteins IFT-81 and IFT-74 promote anterograde IFT of tubulin towards the ciliary tip during axoneme assembly 61,”
From Cellular signalling by primary cilia in development, organ function and disease (2019) · cites this paper as [61]
The Introduction says IFT-B helps transport components into cilia for assembly, and the References list a study of tubulin transport by IFT74 and IFT81. The cited paper does not state that these proteins promote anterograde tubulin transport to the ciliary tip during axoneme assembly; its discussion of IFT81/IFT74 concerns their possible interaction with IFT25/IFT27. (LLM, conf=0.78)
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References 41 · Cited by 260
- Authors
- Eguether, T., San Agustin, J. T., Keady, B. T., Jonassen, J. A., Liang, Y., Francis, R., Tobita, K., Johnson, C. A., Abdelhamed, Z. A., Lo, C. W., & Pazour, G. J.
- Affiliations
- University of Massachusetts Chan Medical School (US)
- University of Pittsburgh (US)
- University of Leeds (GB)
- Corresponding author
- Pazour, Gregory J.
- ORCID
- Venue
- Developmental Cell
- Publisher
- Elsevier BV
- Published
- 2014-11
- Volume
- 31
- Issue
- 3
- Pages
- 279-290
- ISSN
- 1534-5807 (ISSN-L)
- PMC
- PMC4254547
- OpenAlex
- W1980100245
- Cited by
- 260
- References
- 41
- Citations by year
- 2026: 11 · 2025: 19 · 2024: 15 · 2023: 17 · 2022: 22 · 2021: 27 · 2020: 38 · 2019: 21 · 2018: 17 · 2017: 29 · 2016: 25 · 2015: 15 · 2014: 2 · 2013: 1
- FWCI
- 15.54
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Developmental Cell (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- ResearchOnline - JCU (James Cook University) (submittedVersion)
- HAL (submittedVersion)
- Funders
- National Heart, Lung, and Blood Institute (U01HL098180)
- National Institute of General Medical Sciences (GM060992, R01GM060992)
- National Institute of Diabetes and Digestive and Kidney Diseases (DK 074038, DK 32520, P30DK074038, P30DK032520)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Hedgehog Signaling Pathway Studies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Keywords
- BBSome, LZTFL1, intraflagellar transport, primary cilia, Hedgehog signaling, Patched1, Smoothened, structural birth defects
- MeSH terms
- Animals, Biological Transport, Cilia, Flagella, Mice, Inbred C57BL, Mice, Transgenic, Receptors, Cell Surface, Transcription Factors, Signal Transduction, Mice, Knockout, rab GTP-Binding Proteins, Mice, Hedgehog Proteins
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