IFT27 links the BBSome to IFT for maintenance of the ciliary signaling compartment

Eguether, Thibaut, San Agustin, Jovenal T., Keady, Brian T., Jonassen, Julie A., Liang, Yinwen, Francis, Richard, Tobita, Kimimasa, Johnson, Colin A., Abdelhamed, Zakia A., Lo, Cecilia W., and Pazour, Gregory J. (2014) IFT27 links the BBSome to IFT for maintenance of the ciliary signaling compartment. Developmental Cell, 31 (3). pp. 279-290.

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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.

Item ID: 60713
Item Type: Article (Research - C1)
ISSN: 1878-1551
Copyright Information: © 2014 Elsevier Inc.
Funders: NIH, European Community’s Seventh Framework Programme (FP7/2009), China Scholarship Council, Diabetes Endocrinology Research Center (DERC), Alabama Recessive Polycystic Kidney Disease Core Center ((DAB RPKDCC)
Projects and Grants: NIH GM060992, NIH 5U01HL098180, FP7 241955 SYSCILIA, China Scholarship Council no.2011621077, DERC DK32520, DAB RPKDCC DK074038
Date Deposited: 23 Oct 2019 12:36
FoR Codes: 06 BIOLOGICAL SCIENCES > 0604 Genetics > 060408 Genomics @ 35%
06 BIOLOGICAL SCIENCES > 0606 Physiology > 060602 Animal Physiology - Cell @ 30%
06 BIOLOGICAL SCIENCES > 0601 Biochemistry and Cell Biology > 060111 Signal Transduction @ 35%
SEO Codes: 92 HEALTH > 9201 Clinical Health (Organs, Diseases and Abnormal Conditions) > 920199 Clinical Health (Organs, Diseases and Abnormal Conditions) not elsewhere classified @ 100%
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