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Marie-Noëlle Labour, Mathieu Riffault, Søren T Christensen, David A Hoey
The recruitment of mesenchymal stem cells (MSCs) is a crucial process in the development, maintenance and repair of tissues throughout the body. Transforming growth factor-β1 (TGFβ1) is a potent chemokine essential for the recruitment of MSCs in bone, coupling the remodelling cycle. The primary cilium is a sensory organelle with important roles in bone and has been associated with cell migration and more recently TGFβ signalling. Dysregulation of TGFβ signalling or cilia has been linked to a number of skeletal pathologies...
October 17, 2016: Scientific Reports
Miao Yu, Yu Liu, Jing Li, Brianna N Natale, Shuqin Cao, Dongliang Wang, Jeffrey D Amack, Huaiyu Hu
Mutations in the extracellular matrix protein eyes shut homolog (EYS) cause photoreceptor degeneration in patients with retinitis pigmentosa 25. Functions of EYS remain poorly understood, due in part to the lack of an EYS gene in mouse. We investigated the localization of vertebrate EYS proteins and engineered loss-of-function alleles in zebrafish. Immunostaining indicated that EYS localized near the connecting cilium/transition zone in photoreceptors. EYS also strongly localized to the cone outer segments and weakly to the rod outer segments and cone terminals in primate retinas...
October 13, 2016: Biology Open
Erica Sarchielli, Paolo Comeglio, Roberta Squecco, Lara Ballerini, Tommaso Mello, Giulia Guarnieri, Eglantina Idrizaj, Benedetta Mazzanti, Linda Vignozzi, Pasquale Gallina, Mario Maggi, Gabriella B Vannelli, Annamaria Morelli
CONTEXT: Previous studies have suggested that inflammatory pathways may impair central regulatory networks involving gonadotropin-releasing hormone (GnRH) neuron activity. Studies in humans are strongly hampered by the lack of human GnRH neuron cell lines. OBJECTIVE: To establish an in vitro model of GnRH neurons of human origin and analyze the effects of proinflammatory cytokines on their biological properties. Design, samples and treatments: The primary human fetal hypothalamic (hfHypo) cell cultures were isolated from brain of three 12 week-old fetuses...
October 13, 2016: Journal of Clinical Endocrinology and Metabolism
Chia-Yih Wang, Hui-Ling Tsai, Jhih-Siang Syu, Ting-Yu Chen, Mei-Tsz Su
Trophoblast invasion is an important event in embryo implantation and placental development. During these processes, endocrine gland-derived vascular endothelial growth factor (EG-VEGF) is the key regulator mediating the crosstalk at the feto-maternal interface. The primary cilium is a cellular antenna receiving environmental signals and is crucial for proper development. However, little is known regarding the role of the primary cilium in early human pregnancy. Here, we demonstrate that EG-VEGF regulates trophoblast cell invasion via primary cilia...
October 13, 2016: Journal of Cellular Physiology
Je Yeong Ko
The primary cilium is a microtubule-based organelle that is considered to be a cellular antennae, because proteins related to multiple signaling pathways such as Wnt, PDGFRα, Hh, and mechanosignaling are localized to the membrane of the primary cilium. In the kidney, primary cilia extend from the cell membrane to the lumen of renal tubules to respond to fluidic stress. Recent studies have indicated that the disruption of ciliary proteins including polycystin-1 (PC1), polycystin-2 (PC2), and members of the intraflagellar transport (IFT) family induce the development of polycystic kidney disease (PKD), suggesting that the malformation or absence of primary cilia is a driving force of the onset of PKD...
2016: Advances in Experimental Medicine and Biology
Do Yeon Kim, Jong Hoon Park
Autosomal dominant polycystic kidney disease is caused by mutation of PKD1 (polycystic kidney disease-1) or PKD2 (polycystic kidney disease-2). PKD1 and PKD2 encode PC1 (polycystin-1) and PC2 (polycystin-2), respectively. In addition, the mutation of cilia-associated proteins is also a recognized major factor of pathogenesis, since PC1 and PC2 are located in primary cilium. Abnormalities of PC1 or PC2 lead to aberrant signaling through downstream pathways, such as the negative growth regulation, G protein activation, and canonical and non-canonical Wnt pathways...
2016: Advances in Experimental Medicine and Biology
Chen-Jei Hong, Bruce A Hamilton
Zfp423 encodes a 30-zinc finger transcription factor that intersects several canonical signaling pathways. Zfp423 mutations result in ciliopathy-related phenotypes, including agenesis of the cerebellar vermis in mice and Joubert syndrome (JBTS19) and nephronophthisis (NPHP14) in humans. Unlike most ciliopathy genes, Zfp423 encodes a nuclear protein and its developmental expression is complex, leading to alternative proposals for cellular mechanisms. Here we show that Zfp423 is expressed by cerebellar granule cell precursors, that loss of Zfp423 in these precursors leads to cell-intrinsic reduction in proliferation, loss of response to Shh, and primary cilia abnormalities that include diminished frequency of both Smoothened and IFT88 localization...
October 2016: PLoS Genetics
Thomas Cavalier-Smith, Ema E Chao, Keith Vickerman
We describe three new phagotrophic euglenoid species by light microscopy and 18S rDNA and Hsp90 sequencing: Scytomonas saepesedens; Decastava edaphica; Entosiphon oblongum. We studied Scytomonas and Decastava ultrastructure. Scytomonas saepesedens feeds when sessile with actively beating cilium, and has five pellicular strips with flush joints and Calycimonas-like microtubule-supported cytopharynx. Decastava, sister to Keelungia forming new clade Decastavida on 18S rDNA trees, has 10 broad strips with cusp-like joints, not bifurcate ridges like Ploeotia and Serpenomonas (phylogenetically and cytologically distinct genera), and Serpenomonas-like feeding apparatus (8-9 unreinforced microtubule pairs loop from dorsal jaw support to cytostome)...
August 28, 2016: European Journal of Protistology
Maleeha Maria, Ideke J C Lamers, Miriam Schmidts, Muhammad Ajmal, Sulman Jaffar, Ehsan Ullah, Bilal Mustafa, Shakeel Ahmad, Katia Nazmutdinova, Bethan Hoskins, Erwin van Wijk, Linda Koster-Kamphuis, Muhammad Imran Khan, Phil L Beales, Frans P M Cremers, Ronald Roepman, Maleeha Azam, Heleen H Arts, Raheel Qamar
Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder that is both genetically and clinically heterogeneous. To date 19 genes have been associated with BBS, which encode proteins active at the primary cilium, an antenna-like organelle that acts as the cell's signaling hub. In the current study, a combination of mutation screening, targeted sequencing of ciliopathy genes associated with BBS, and whole-exome sequencing was used for the genetic characterization of five families including four with classic BBS symptoms and one BBS-like syndrome...
October 6, 2016: Scientific Reports
Sukanya Lodh, Junji Yano, Megan S Valentine, Judith L Van Houten
Paramecium cells swim by beating their cilia, and make turns by transiently reversing their power stroke. Reversal is caused by Ca(2+) entering the cilium through voltage-gated Ca(2+) (CaV) channels that are found exclusively in the cilia. As ciliary Ca(2+) levels return to normal, the cell pivots and swims forward in a new direction. Thus, the activation of the CaV channels causes cells to make a turn in their swimming paths. For 45 years, the physiological characteristics of the Paramecium ciliary CaV channels have been known, but the proteins were not identified until recently, when the P...
October 1, 2016: Journal of Experimental Biology
Celine Kempeneers, Claire Seaton, Mark A Chilvers
BACKGROUND: Digital high speed videomicroscopy(DHSV) allows analysis of ciliary beat frequency(CBF) and pattern(CBP) of respiratory cilia in 3 planes. Normal reference data use a sideways edge to evaluate ciliary dyskinesia, and calculate CBF using the time for a cilium to complete 10 beat cycles. Variability in CBF within respiratory epithelium has been described, but data concerning variation of CBP is limited in healthy epithelium. This study aims to document variability of CBP within normal samples, to compare ciliary function in 3 profiles, and CBF calculated over 5 or 10 beat cycles...
September 29, 2016: Chest
Doudou Wang, Ryo Nitta, Manatsu Morikawa, Hiroaki Yajima, Shigeyuki Inoue, Hideki Shigematsu, Masahide Kikkawa, Nobutaka Hirokawa
The kinesin-8 motor, KIF19A, accumulates at cilia tips and controls cilium length. Defective KIF19A leads to hydrocephalus and female infertility because of abnormally elongated cilia. Uniquely among kinesins, KIF19A possesses the dual functions of motility along ciliary microtubules and depolymerization of microtubules. To elucidate the molecular mechanisms of these functions we solved the crystal structure of its motor domain and determined its cryo-electron microscopy structure complexed with a microtubule...
September 30, 2016: ELife
Laura E Mariani, Maarten F Bijlsma, Anna I Ivanova, Sarah K Suciu, Richard A Kahn, Tamara Caspary
The regulatory GTPase Arl13b localizes to primary cilia, where it regulates Sonic hedgehog (Shh) signaling. Missense mutations in ARL13B can cause the ciliopathy Joubert syndrome, while the mouse null allele is embryonic lethal. We used mouse embryonic fibroblasts as a system to determine the effects of Arl13b mutations on Shh signaling. We tested a total of seven different mutants, three JS-causing variants, two point mutants predicted to alter guanine nucleotide handling, one that disrupts cilia localization, and one that prevents palmitoylation and thus membrane binding, in assays of transcriptional and non-transcriptional Shh signaling...
September 28, 2016: Molecular Biology of the Cell
Soyoung Rhee, Gregory W Kirschen, Yan Gu, Shaoyu Ge
The primary cilium, a sensory organelle, regulates cell proliferation and neuronal development of dentate granule cells in the hippocampus. However, its role in the function of mature dentate granule cells remains unknown. Here we specifically depleted and disrupted ciliary proteins IFT20 and Kif3A (respectively) in mature dentate granule cells and investigated hippocampus-dependent contextual memory and long-term plasticity at mossy fiber synapses. We found that depletion of IFT20 in these cells significantly impaired context-dependent fear-related memory...
September 28, 2016: Scientific Reports
Guo Zhan Lum, Zhou Ye, Xiaoguang Dong, Hamid Marvi, Onder Erin, Wenqi Hu, Metin Sitti
Shape-programmable matter is a class of active materials whose geometry can be controlled to potentially achieve mechanical functionalities beyond those of traditional machines. Among these materials, magnetically actuated matter is particularly promising for achieving complex time-varying shapes at small scale (overall dimensions smaller than 1 cm). However, previous work can only program these materials for limited applications, as they rely solely on human intuition to approximate the required magnetization profile and actuating magnetic fields for their materials...
October 11, 2016: Proceedings of the National Academy of Sciences of the United States of America
Hidemasa Goto, Hironori Inaba, Masaki Inagaki
The primary cilium is a non-motile and microtubule-enriched protrusion ensheathed by plasma membrane. Primary cilia function as mechano/chemosensors and signaling hubs and their disorders predispose to a wide spectrum of human diseases. Most types of cells assemble their primary cilia in response to cellular quiescence, whereas they start to retract the primary cilia upon cell-cycle reentry. The retardation of ciliary resorption process has been shown to delay cell-cycle progression to the S or M phase after cell-cycle reentry...
September 26, 2016: Cellular and Molecular Life Sciences: CMLS
Ivan Duran, S Paige Taylor, Wenjuan Zhang, Jorge Martin, Kimberly N Forlenza, Rhonda P Spiro, Deborah A Nickerson, Michael Bamshad, Daniel H Cohn, Deborah Krakow
Short-rib polydactyly syndromes (SRPS) and Asphyxiating thoracic dystrophy (ATD) or Jeune Syndrome are recessively inherited skeletal ciliopathies characterized by profound skeletal abnormalities and are frequently associated with polydactyly and multiorgan system involvement. SRPS are produced by mutations in genes that participate in the formation and function of primary cilia and usually result from disruption of retrograde intraflagellar (IFT) transport of the cilium. Herein we describe a new spectrum of SRPS caused by mutations in the gene IFT81, a key component of the IFT-B complex essential for anterograde transport...
September 26, 2016: Scientific Reports
Yuriko Minegishi, XunLun Sheng, Kazutoshi Yoshitake, Yuri Sergeev, Daisuke Iejima, Yoshio Shibagaki, Norikazu Monma, Kazuho Ikeo, Masaaki Furuno, Wenjun Zhuang, Yani Liu, Weining Rong, Seisuke Hattori, Takeshi Iwata
Leber congenital amaurosis (LCA) is a hereditary early-onset retinal dystrophy that is accompanied by severe macular degeneration. In this study, novel compound heterozygous mutations were identified as LCA-causative in chaperonin-containing TCP-1, subunit 2 (CCT2), a gene that encodes the molecular chaperone protein, CCTβ. The zebrafish mutants of CCTβ are known to exhibit the eye phenotype while its mutation and association with human disease have been unknown. The CCT proteins (CCT α-θ) forms ring complex for its chaperon function...
2016: Scientific Reports
Jarema J Malicki, Colin A Johnson
Cilia mediate an astonishing diversity of processes. Recent advances provide unexpected insights into the regulatory mechanisms of cilium formation, and reveal diverse regulatory inputs that are related to the cell cycle, cytoskeleton, proteostasis, and cilia-mediated signaling itself. Ciliogenesis and cilia maintenance are regulated by reciprocal antagonistic or synergistic influences, often acting in parallel to each other. By receiving parallel inputs, cilia appear to integrate multiple signals into specific outputs and may have functions similar to logic gates of digital systems...
September 12, 2016: Trends in Cell Biology
Inna V Nechipurenko, Anique Olivier-Mason, Anna Kazatskaya, Julie Kennedy, Ian G McLachlan, Maxwell G Heiman, Oliver E Blacque, Piali Sengupta
Primary cilia are ubiquitous sensory organelles that mediate diverse signaling pathways. Cilia position on the cell surface is determined by the location of the basal body (BB) that templates the cilium. The mechanisms that regulate BB positioning in the context of ciliogenesis are largely unknown. Here we show that the conserved signaling and scaffolding protein Girdin localizes to the proximal regions of centrioles and regulates BB positioning and ciliogenesis in Caenorhabditis elegans sensory neurons and human RPE-1 cells...
September 12, 2016: Developmental Cell
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