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https://www.readbyqxmd.com/read/27519801/cilium-transition-zone-proteome-reveals-compartmentalization-and-differential-dynamics-of-ciliopathy-complexes
#1
Samuel Dean, Flavia Moreira-Leite, Vladimir Varga, Keith Gull
The transition zone (TZ) of eukaryotic cilia and flagella is a structural intermediate between the basal body and the axoneme that regulates ciliary traffic. Mutations in genes encoding TZ proteins (TZPs) cause human inherited diseases (ciliopathies). Here, we use the trypanosome to identify TZ components and localize them to TZ subdomains, showing that the Bardet-Biedl syndrome complex (BBSome) is more distal in the TZ than the Meckel syndrome (MKS) complex. Several of the TZPs identified here have human orthologs...
August 30, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27393972/the-intraflagellar-transport-machinery-in-ciliary-signaling
#2
André Mourão, Søren T Christensen, Esben Lorentzen
Cilia and flagella on eukaryotic cells are slender microtubule-based projections surrounded by a membrane with a unique lipid and protein composition. It is now appreciated that cilia in addition to their established roles in motility also constitute hubs for cellular signaling by sensing external environmental cues necessary for organ development and maintenance of human health. Pathways reported to rely on the cilium organelle include Hedgehog, TGF-β, Wnt, PDGFRα, integrin and DNA damage repair signaling...
July 6, 2016: Current Opinion in Structural Biology
https://www.readbyqxmd.com/read/27149508/hypothalamic-leptin-resistance-from-bbb-to-bbsome
#3
Miguel López
No abstract text is available yet for this article.
May 2016: PLoS Genetics
https://www.readbyqxmd.com/read/26980730/overall-architecture-of-the-intraflagellar-transport-ift-b-complex-containing-cluap1-ift38-as-an-essential-component-of-the-ift-b-peripheral-subcomplex
#4
Yohei Katoh, Masaya Terada, Yuya Nishijima, Ryota Takei, Shohei Nozaki, Hiroshi Hamada, Kazuhisa Nakayama
Intraflagellar transport (IFT) is essential for assembly and maintenance of cilia and flagella as well as ciliary motility and signaling. IFT is mediated by multisubunit complexes, including IFT-A, IFT-B, and the BBSome, in concert with kinesin and dynein motors. Under high salt conditions, purified IFT-B complex dissociates into a core subcomplex composed of at least nine subunits and at least five peripherally associated proteins. Using the visible immunoprecipitation assay, which we recently developed as a convenient protein-protein interaction assay, we determined the overall architecture of the IFT-B complex, which can be divided into core and peripheral subcomplexes composed of 10 and 6 subunits, respectively...
May 20, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/26926121/the-bbsome-controls-energy-homeostasis-by-mediating-the-transport-of-the-leptin-receptor-to-the-plasma-membrane
#5
Deng-Fu Guo, Huxing Cui, Qihong Zhang, Donald A Morgan, Daniel R Thedens, Darryl Nishimura, Justin L Grobe, Val C Sheffield, Kamal Rahmouni
Bardet-Biedl syndrome (BBS) is a highly pleiotropic autosomal recessive disorder associated with a wide range of phenotypes including obesity. However, the underlying mechanism remains unclear. Here, we show that neuronal BBSome is a critical determinant of energy balance through its role in the regulation of the trafficking of the long signaling form of the leptin receptor (LRb). Targeted disruption of the BBSome by deleting the Bbs1 gene from the nervous system causes obesity in mice, and this phenotype is reproduced by ablation of the Bbs1 gene selectively in the LRb-expressing cells, but not from adipocytes...
February 2016: PLoS Genetics
https://www.readbyqxmd.com/read/26867008/a-splice-variant-of-bardet-biedl-syndrome-5-bbs5-protein-that-is-selectively-expressed-in-retina
#6
Susan N Bolch, Donald R Dugger, Timothy Chong, J Hugh McDowell, W Clay Smith
PURPOSE: Bardet-Biedl syndrome is a complex ciliopathy that usually manifests with some form of retinal degeneration, amongst other ciliary-related deficiencies. One of the genetic causes of this syndrome results from a defect in Bardet-Biedl Syndrome 5 (BBS5) protein. BBS5 is one component of the BBSome, a complex of proteins that regulates the protein composition in cilia. In this study, we identify a smaller molecular mass form of BBS5 as a variant formed by alternative splicing and show that expression of this splice variant is restricted to the retina...
2016: PloS One
https://www.readbyqxmd.com/read/26721397/loss-of-the-bbsome-perturbs-endocytic-trafficking-and-disrupts-virulence-of-trypanosoma-brucei
#7
Gerasimos Langousis, Michelle M Shimogawa, Edwin A Saada, Ajay A Vashisht, Roberto Spreafico, Andrew R Nager, William D Barshop, Maxence V Nachury, James A Wohlschlegel, Kent L Hill
Cilia (eukaryotic flagella) are present in diverse eukaryotic lineages and have essential motility and sensory functions. The cilium's capacity to sense and transduce extracellular signals depends on dynamic trafficking of ciliary membrane proteins. This trafficking is often mediated by the Bardet-Biedl Syndrome complex (BBSome), a protein complex for which the precise subcellular distribution and mechanisms of action are unclear. In humans, BBSome defects perturb ciliary membrane protein distribution and manifest clinically as Bardet-Biedl Syndrome...
January 19, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/26654377/shared-and-distinct-mechanisms-of-compartmentalized-and-cytosolic-ciliogenesis
#8
REVIEW
Tomer Avidor-Reiss, Michel R Leroux
Most motile and all non-motile (also known as primary) eukaryotic cilia possess microtubule-based axonemes that are assembled at the cell surface to form hair-like or more elaborate compartments endowed with motility and/or signaling functions. Such compartmentalized ciliogenesis depends on the core intraflagellar transport (IFT) machinery and the associated Bardet-Biedl syndrome complex (BBSome) for dynamic delivery of ciliary components. The transition zone (TZ), an ultrastructurally complex barrier or 'gate' at the base of cilia, also contributes to the formation of compartmentalized cilia...
December 7, 2015: Current Biology: CB
https://www.readbyqxmd.com/read/26585297/proteomics-of-primary-cilia-by-proximity-labeling
#9
David U Mick, Rachel B Rodrigues, Ryan D Leib, Christopher M Adams, Allis S Chien, Steven P Gygi, Maxence V Nachury
While cilia are recognized as important signaling organelles, the extent of ciliary functions remains unknown because of difficulties in cataloguing proteins from mammalian primary cilia. We present a method that readily captures rapid snapshots of the ciliary proteome by selectively biotinylating ciliary proteins using a cilia-targeted proximity labeling enzyme (cilia-APEX). Besides identifying known ciliary proteins, cilia-APEX uncovered several ciliary signaling molecules. The kinases PKA, AMPK, and LKB1 were validated as bona fide ciliary proteins and PKA was found to regulate Hedgehog signaling in primary cilia...
November 23, 2015: Developmental Cell
https://www.readbyqxmd.com/read/26540106/conserved-genetic-interactions-between-ciliopathy-complexes-cooperatively-support-ciliogenesis-and-ciliary-signaling
#10
Laura E Yee, Francesc R Garcia-Gonzalo, Rachel V Bowie, Chunmei Li, Julie K Kennedy, Kaveh Ashrafi, Oliver E Blacque, Michel R Leroux, Jeremy F Reiter
Mutations in genes encoding cilia proteins cause human ciliopathies, diverse disorders affecting many tissues. Individual genes can be linked to ciliopathies with dramatically different phenotypes, suggesting that genetic modifiers may participate in their pathogenesis. The ciliary transition zone contains two protein complexes affected in the ciliopathies Meckel syndrome (MKS) and nephronophthisis (NPHP). The BBSome is a third protein complex, affected in the ciliopathy Bardet-Biedl syndrome (BBS). We tested whether mutations in MKS, NPHP and BBS complex genes modify the phenotypic consequences of one another in both C...
November 2015: PLoS Genetics
https://www.readbyqxmd.com/read/26150102/bbs4-and-bbs5-show-functional-redundancy-in-the-bbsome-to-regulate-the-degradative-sorting-of-ciliary-sensory-receptors
#11
Qingwen Xu, Yuxia Zhang, Qing Wei, Yan Huang, Yan Li, Kun Ling, Jinghua Hu
Cilia harbor sensory receptors for various signaling cascades critical for vertebrate development. However, the mechanisms underlying the ciliary homeostasis of sensory receptors remain elusive. Here, we demonstrate that BBS-4 and BBS-5, two distinct BBSome components, show unexpected functional redundancy in the context of cilia in C. elegans. BBS-4 directly interacts with BBS-5 and the interaction can be disrupted by a conserved mutation identified in human BBS4. Surprisingly, we found that BBS-4 and BBS-5 act redundantly in the BBSome to regulate the ciliary removal, rather than the ciliary entry or retrograde IFT transport, of various sensory receptors...
2015: Scientific Reports
https://www.readbyqxmd.com/read/26085087/structural-characterization-of-bardet-biedl-syndrome-9-protein-bbs9
#12
Kevin E Knockenhauer, Thomas U Schwartz
The Bardet-Biedl syndrome protein complex (BBSome) is an octameric complex that transports membrane proteins into the primary cilium signaling organelle in eukaryotes and is implicated in human disease. Here we have analyzed the 99-kDa human BBS9 protein, one of the eight BBSome components. The protein is composed of four structured domains, including a β-stranded N-terminal domain. The 1.8 Å crystal structure of the 46-kDa N-terminal domain reveals a seven-bladed β-propeller. A structure-based homology search suggests that it functions in protein-protein interactions...
August 7, 2015: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/26051893/assembly-of-ift-trains-at-the-ciliary-base-depends-on-ift74
#13
Jason M Brown, Deborah A Cochran, Branch Craige, Tomohiro Kubo, George B Witman
Intraflagellar transport (IFT) moves IFT trains carrying cargoes from the cell body into the flagellum and from the flagellum back to the cell body. IFT trains are composed of complexes IFT-A and IFT-B and cargo adaptors such as the BBSome. The IFT-B core proteins IFT74 and IFT81 interact directly through central and C-terminal coiled-coil domains, and recently it was shown that the N termini of these proteins form a tubulin-binding module important for ciliogenesis. To investigate the function of IFT74 and its domains in vivo, we have utilized Chlamydomonas reinhardtii ift74 mutants...
June 15, 2015: Current Biology: CB
https://www.readbyqxmd.com/read/25964651/architectures-of-multisubunit-complexes-revealed-by-a-visible-immunoprecipitation-assay-using-fluorescent-fusion-proteins
#14
Yohei Katoh, Shohei Nozaki, David Hartanto, Rie Miyano, Kazuhisa Nakayama
In this study, we elucidated the architectures of two multisubunit complexes, the BBSome and exocyst, through a novel application of fluorescent fusion proteins. By processing lysates from cells co-expressing GFP and RFP fusion proteins for immunoprecipitation with anti-GFP nanobody, protein-protein interactions could be reproducibly visualized by directly observing the immunoprecipitates under a microscope, and evaluated using a microplate reader, without requiring immunoblotting. Using this 'visible' immunoprecipitation (VIP) assay, we mapped binary subunit interactions of the BBSome complex, and determined the hierarchies of up to four subunit interactions...
June 15, 2015: Journal of Cell Science
https://www.readbyqxmd.com/read/25860617/characterization-of-tetratricopeptide-repeat-containing-proteins-critical-for-cilia-formation-and-function
#15
Yanan Xu, Jingli Cao, Shan Huang, Di Feng, Wei Zhang, Xueliang Zhu, Xiumin Yan
Cilia formation and function require a special set of trafficking machinery termed intraflagellar transport (IFT), consisting mainly of protein complexes IFT-A, IFT-B, BBSome, and microtubule-dependent molecular motors. Tetratricopeptide repeat-containing (TTC) proteins are widely involved in protein complex formation. Nine of them are known to serve as components of the IFT or BBSome complexes. How many TTC proteins are cilia-related and how they function, however, remain unclear. Here we show that twenty TTC genes were upregulated by at least 2-fold during the differentiation of cultured mouse tracheal epithelial cells (MTECs) into multiciliated cells...
2015: PloS One
https://www.readbyqxmd.com/read/25552655/nephrocystin-proteins-nphp5-and-cep290-regulate-bbsome-integrity-ciliary-trafficking-and-cargo-delivery
#16
Marine Barbelanne, Delowar Hossain, David Puth Chan, Johan Peränen, William Y Tsang
Proper functioning of cilia, hair-like structures responsible for sensation and locomotion, requires nephrocystin-5 (NPHP5) and a multi-subunit complex called the Bardet-Biedl syndrome (BBS)ome, but their precise relationship is not understood. The BBSome is involved in the trafficking of membrane cargos to cilia. While it is known that a loss of any single subunit prevents ciliary trafficking of the BBSome and its cargos, the mechanisms underlying ciliary entry of this complex are not well characterized. Here, we report that a transition zone protein NPHP5 contains two separate BBS-binding sites and interacts with the BBSome to mediate its integrity...
April 15, 2015: Human Molecular Genetics
https://www.readbyqxmd.com/read/25504142/direct-evidence-for-bbsome-associated-intraflagellar-transport-reveals-distinct-properties-of-native-mammalian-cilia
#17
Corey L Williams, Jeremy C McIntyre, Stephen R Norris, Paul M Jenkins, Lian Zhang, Qinglin Pei, Kristen Verhey, Jeffrey R Martens
Cilia dysfunction underlies a class of human diseases with variable penetrance in different organ systems. Across eukaryotes, intraflagellar transport (IFT) facilitates cilia biogenesis and cargo trafficking, but our understanding of mammalian IFT is insufficient. Here we perform live analysis of cilia ultrastructure, composition and cargo transport in native mammalian tissue using olfactory sensory neurons. Proximal and distal axonemes of these neurons show no bias towards IFT kinesin-2 choice, and Kif17 homodimer is dispensable for distal segment IFT...
December 15, 2014: Nature Communications
https://www.readbyqxmd.com/read/25446516/ift27-links-the-bbsome-to-ift-for-maintenance-of-the-ciliary-signaling-compartment
#18
Thibaut Eguether, Jovenal T San Agustin, Brian T Keady, Julie A Jonassen, Yinwen Liang, Richard Francis, Kimimasa Tobita, Colin A Johnson, Zakia A Abdelhamed, Cecilia W Lo, Gregory J Pazour
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...
November 10, 2014: Developmental Cell
https://www.readbyqxmd.com/read/25443296/the-intraflagellar-transport-protein-ift27-promotes-bbsome-exit-from-cilia-through-the-gtpase-arl6-bbs3
#19
Gerald M Liew, Fan Ye, Andrew R Nager, J Patrick Murphy, Jaclyn S Lee, Mike Aguiar, David K Breslow, Steven P Gygi, Maxence V Nachury
The sorting of signaling receptors into and out of cilia relies on the BBSome, a complex of Bardet-Biedl syndrome (BBS) proteins, and on the intraflagellar transport (IFT) machinery. GTP loading onto the Arf-like GTPase ARL6/BBS3 drives assembly of a membrane-apposed BBSome coat that promotes cargo entry into cilia, yet how and where ARL6 is activated remains elusive. Here, we show that the Rab-like GTPase IFT27/RABL4, a known component of IFT complex B, promotes the exit of BBSome and associated cargoes from cilia...
November 10, 2014: Developmental Cell
https://www.readbyqxmd.com/read/25402481/structural-basis-for-membrane-targeting-of-the-bbsome-by-arl6
#20
André Mourão, Andrew R Nager, Maxence V Nachury, Esben Lorentzen
The BBSome is a coat-like ciliary trafficking complex composed of proteins mutated in Bardet-Biedl syndrome (BBS). A critical step in BBSome-mediated sorting is recruitment of the BBSome to membranes by the GTP-bound Arf-like GTPase ARL6. We have determined crystal structures of Chlamydomonas reinhardtii ARL6-GDP, ARL6-GTP and the ARL6-GTP-BBS1 complex. The structures demonstrate how ARL6-GTP binds the BBS1 β-propeller at blades 1 and 7 and explain why GTP- but not GDP-bound ARL6 can recruit the BBSome to membranes...
December 2014: Nature Structural & Molecular Biology
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