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https://www.readbyqxmd.com/read/28346501/a-tissue-specific-role-for-intraflagellar-transport-genes-during-craniofacial-development
#1
Elizabeth N Schock, Jaime N Struve, Ching-Fang Chang, Trevor J Williams, John Snedeker, Aria C Attia, Rolf W Stottmann, Samantha A Brugmann
Primary cilia are nearly ubiquitous, cellular projections that function to transduce molecular signals during development. Loss of functional primary cilia has a particularly profound effect on the developing craniofacial complex, causing several anomalies including craniosynostosis, micrognathia, midfacial dysplasia, cleft lip/palate and oral/dental defects. Development of the craniofacial complex is an intricate process that requires interactions between several different tissues including neural crest cells, neuroectoderm and surface ectoderm...
2017: PloS One
https://www.readbyqxmd.com/read/28342295/intraflagellar-transport-mechanisms-of-motor-action-cooperation-and-cargo-delivery
#2
REVIEW
Bram Prevo, Jonathan M Scholey, Erwin J G Peterman
Intraflagellar transport (IFT) is a form of motor-dependent cargo transport that is essential for the assembly, maintenance and length-control of cilia, which play critical roles in motility, sensory reception and signal transduction in virtually all eukaryotic cells. During IFT, anterograde kinesin-2 and retrograde IFT-dynein motors drive the bidirectional transport of IFT trains that deliver cargo, for example axoneme precursors such as tubulins as well as molecules of the signal transduction machinery, to their site of assembly within the cilium...
March 25, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28332184/primary-cilium-alterations-and-expression-changes-of-patched1-proteins-in-niemann-pick-type-c-disease
#3
Patrizia Formichi, Carla Battisti, Maria Margherita De Santi, Raffaella Guazzo, Sergio Antonio Tripodi, Elena Radi, Benedetta Rossi, Ermelinda Tarquini, Antonio Federico
Niemann-Pick type C disease (NPC) is a disorder characterized by abnormal intracellular accumulation of unesterified cholesterol and glycolipids. Two distinct disease-causing genes have been isolated, NPC1 and NPC2. The NPC1 protein is involved in the sorting and recycling of cholesterol and glycosphingolipids in the late endosomal/lysosomal system. It has extensive homology with the Patched 1 (Ptc1) receptor, a transmembrane protein localized in the primary cilium and involved in the Hedgehog signaling (Shh) pathway...
March 23, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28320755/ciliary-mechanisms-of-cyst-formation-in-polycystic-kidney-disease
#4
Ming Ma, Anna-Rachel Gallagher, Stefan Somlo
Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled sacs called cysts. The causal genes PKD1 and PKD2 encode transmembrane proteins polycystin 1 (PC1) and polycystin 2 (PC2), respectively. Together, the polycystins localize to the solitary primary cilium that protrudes from the apical surface of most kidney tubule cells and is thought to function as a privileged compartment that the cell uses for signal integration of sensory inputs...
March 20, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28319439/loss-of-a-primary-cilium-in-pdac
#5
Tetsuo Kobayashi, Hiroshi Itoh
No abstract text is available yet for this article.
March 20, 2017: Cell Cycle
https://www.readbyqxmd.com/read/28298612/cell-scientist-to-watch-gaia-pigino
#6
(no author information available yet)
Gaia Pigino received her master's degree in natural science followed by a PhD in evolutionary biology - focusing on zoology and ecotoxicology - in the laboratory of Fabio Bernini at the University of Siena, Italy. She then joined the electron microscopy laboratory of Pietro Lupetti for her first postdoctoral position before moving to Zürich. There, supported by an EMBO Long-Term Fellowship, she worked with Takashi Ishikawa at the Swiss Federal Institute of Technology and the Paul Scherrer Institute in Villigen...
March 15, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28295209/a-nonsense-mutation-in-cep55-defines-a-new-locus-for-a-meckel-like-syndrome-an-autosomal-recessive-lethal-fetal-ciliopathy
#7
Marie-Louise Bondeson, Katharina Ericson, Sanna Gudmundsson, Adam Ameur, Fredrik Pontén, Jan Wesström, Carina Frykholm, Maria Wilbe
Mutations in genes involved in the cilium-centrosome complex are called ciliopathies. Meckel-Gruber syndrome (MKS) is a ciliopathic lethal autosomal recessive syndrome characterized by genetically and clinically heterogeneous manifestations, including renal cystic dysplasia, occipital encephalocele and polydactyly. Several genes have previously been associated with MKS and MKS-like phenotypes, but there are still genes remaining to be discovered. We have used whole exome sequencing (WES) to uncover the genetics of a suspected autosomal recessive Meckel syndrome phenotype in a family with two affected fetuses...
March 14, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28291812/characterization-of-a-new-oda3-allele-oda3-6-defective-in-assembly-of-the-outer-dynein-arm-docking-complex-in-chlamydomonas-reinhardtii
#8
Jason M Brown, Matthew Mosley, Daniela Montes-Berrueta, Yuqing Hou, Fan Yang, Chasity Scarbrough, George B Witman, Maureen Wirschell
We have used an insertional mutagenesis approach to generate new C. reinhardtii motility mutants. Of 56 mutants isolated, one is a new allele at the ODA3 locus, called oda3-6. Similar to the previously characterized oda3 alleles, oda3-6 has a slow-jerky swimming phenotype and reduced swimming speed. The oda3-6 mutant fails to assemble the outer dynein arm motor and outer dynein arm-docking complex (ODA-DC) in the ciliary axoneme due to an insertion in the 5' end of the DCC1 gene, which encodes the DC1 subunit of the ODA-DC...
2017: PloS One
https://www.readbyqxmd.com/read/28291807/the-meckel-syndrome-associated-protein-mks1-functionally-interacts-with-components-of-the-bbsome-and-ift-complexes-to-mediate-ciliary-trafficking-and-hedgehog-signaling
#9
Sarah C Goetz, Fiona Bangs, Chloe L Barrington, Nicholas Katsanis, Kathryn V Anderson
The importance of primary cilia in human health is underscored by the link between ciliary dysfunction and a group of primarily recessive genetic disorders with overlapping clinical features, now known as ciliopathies. Many of the proteins encoded by ciliopathy-associated genes are components of a handful of multi-protein complexes important for the transport of cargo to the basal body and/or into the cilium. A key question is whether different complexes cooperate in cilia formation, and whether they participate in cilium assembly in conjunction with intraflagellar transport (IFT) proteins...
2017: PloS One
https://www.readbyqxmd.com/read/28290481/identification-of-elongated-primary-cilia-with-impaired-mechanotransduction-in-idiopathic-scoliosis-patients
#10
Niaz Oliazadeh, Kristen F Gorman, Robert Eveleigh, Guillaume Bourque, Alain Moreau
The primary cilium is an outward projecting antenna-like organelle with an important role in bone mechanotransduction. The capacity to sense mechanical stimuli can affect important cellular and molecular aspects of bone tissue. Idiopathic scoliosis (IS) is a complex pediatric disease of unknown cause, defined by abnormal spinal curvatures. We demonstrate significant elongation of primary cilia in IS patient bone cells. In response to mechanical stimulation, these IS cells differentially express osteogenic factors, mechanosensitive genes, and signaling genes...
March 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28289724/flagellar-synchronization-is-a-simple-alternative-to-cell-cycle-synchronization-for-ciliary-and-flagellar-studies
#11
Soumita Dutta, Prachee Avasthi
The unicellular green alga Chlamydomonas reinhardtii is an ideal model organism for studies of ciliary function and assembly. In assays for biological and biochemical effects of various factors on flagellar structure and function, synchronous culture is advantageous for minimizing variability. Here, we have characterized a method in which 100% synchronization is achieved with respect to flagellar length but not with respect to the cell cycle. The method requires inducing flagellar regeneration by amputation of the entire cell population and limiting regeneration time...
March 2017: MSphere
https://www.readbyqxmd.com/read/28286175/cilia-dependent-gli-processing-in-neural-crest-cells-is-required-for-tongue-development
#12
Grethel Millington, Kelsey Elliott, Ya-Ting Chang, Ching-Fang Chang, Andrzej Dlugosz, Samantha A Brugmann
Ciliopathies are a class of diseases caused by the loss of a ubiquitous, microtubule-based organelle called a primary cilium. Ciliopathies commonly result in defective development of the craniofacial complex, causing midfacial defects, craniosynostosis, micrognathia and aglossia. Herein, we explored how the conditional loss of primary cilia on neural crest cells (Kif3a(f/f);Wnt1-Cre) generated aglossia. On a cellular level, our data revealed that aglossia in Kif3a(f/f);Wnt1-Cre embryos was due to a loss of mesoderm-derived muscle precursors migrating into and surviving in the tongue anlage...
March 9, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28271209/tgf-%C3%AE-1-impairs-mechanosensation-of-human-osteoblasts-via-hdac6-mediated-shortening-and-distortion-of-primary-cilia
#13
Sabrina Ehnert, Vrinda Sreekumar, Romina H Aspera-Werz, Sahar O Sajadian, Elke Wintermeyer, Gunther H Sandmann, Christian Bahrs, Jan G Hengstler, Patricio Godoy, Andreas K Nussler
Transforming growth factor β (TGF-β) is a critical regulator of bone density owing to its multiple effects on cell growth and differentiation. Recently, we have shown that TGF-β1 effectively blocks bone morphogenetic protein (BMP) induced maturation of osteoblasts by upregulating histone deacetylase (HDAC) activity. The current study aimed at investigating the effect of rhTGF-β1 treatment on the expression of specific HDACs and their cellular effects, e.g., microtubule structures (primary cilia) and mechanosensation...
March 7, 2017: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/28266547/the-molecular-motor-myosin-va-interacts-with-the-cilia-centrosomal-protein-rpgrip1l
#14
L H P Assis, R M P Silva-Junior, L G Dolce, M R Alborghetti, R V Honorato, A F Z Nascimento, T D Melo-Hanchuk, D M Trindade, C C C Tonoli, C T Santos, P S L Oliveira, R E Larson, J Kobarg, E M Espreafico, P O Giuseppe, M T Murakami
Myosin Va (MyoVa) is an actin-based molecular motor abundantly found at the centrosome. However, the role of MyoVa at this organelle has been elusive due to the lack of evidence on interacting partners or functional data. Herein, we combined yeast two-hybrid screen, biochemical studies and cellular assays to demonstrate that MyoVa interacts with RPGRIP1L, a cilia-centrosomal protein that controls ciliary signaling and positioning. MyoVa binds to the C2 domains of RPGRIP1L via residues located near or in the Rab11a-binding site, a conserved site in the globular tail domain (GTD) from class V myosins...
March 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28237838/gene-therapeutic-reversal-of-peripheral-olfactory-impairment-in-bardet-biedl-syndrome
#15
Corey L Williams, Cedric R Uytingco, Warren W Green, Jeremy C McIntyre, Kirill Ukhanov, Arthur D Zimmerman, Dana T Shively, Lian Zhang, Darryl Y Nishimura, Val C Sheffield, Jeffrey R Martens
Olfactory dysfunction is a pervasive but underappreciated health concern that affects personal safety and quality of life. Patients with olfactory dysfunctions have limited therapeutic options, particularly those involving congenital diseases. Bardet-Biedl syndrome (BBS) is one such disorder, where olfactory loss and other symptoms manifest from defective cilium morphology and/or function in various cell types/tissues. Olfactory sensory neurons (OSNs) of BBS mutant mice lack the capacity to build/maintain cilia, rendering the cells incapable of odor detection...
February 22, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28237397/sox17-regulates-cholangiocyte-differentiation-and-acts-as-a-tumor-suppressor-in-cholangiocarcinoma
#16
M Merino-Azpitarte, E Lozano, M J Perugorria, A Esparza-Baquer, O Erice, A Santos-Laso, C J O'Rourke, J B Andersen, R Jiménez-Agüero, A Lacasta, M D'Amato, O Briz, N Jalan-Sakrikar, R C Huebert, K M Thelen, S A Gradilone, A M Aransay, J L Lavín, M G Fernández-Barrena, A Matheu, M Marzioni, G J Gores, L Bujanda, J J G Marin, J M Banales
BACKGROUND & AIMS: Cholangiocarcinoma (CCA) is a biliary malignancy linked to genetic and epigenetic abnormalities, such as hypermethylation of SOX17 promoter. Here, the role of SOX17 in cholangiocyte differentiation and cholangiocarcinogenesis was studied. METHODS: SOX17 expression/function was evaluated along the differentiation of human induced pluripotent stem cells (iPSC) into cholangiocytes, in the dedifferentiation process of normal human cholangiocytes (NHC) in culture and in cholangiocarcinogenesis...
February 22, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28236036/functional-diversity-of-ciliary-proteins-in-bone-development-and-disease
#17
REVIEW
Masaru Kaku, Yoshihiro Komatsu
PURPOSE OF REVIEW: The primary cilium is a non-motile microtubule-based organelle that senses a diverse range of extracellular signals. While recent studies highlight the importance of ciliary-dependent developmental signals, including Hedgehog, Wnt, and platelet-derived growth factor, it is not well understood whether and how bone morphogenetic protein (BMP) signaling, a key regulator of skeletogenesis, is involved in cilia-related bone developmental aspects and in the etiology of skeletal disorders...
February 24, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28235840/centriolar-satellite-protein-ccdc66-interacts-with-cep290-and-functions-in-cilium-formation-and-trafficking
#18
Deniz Conkar, Efraim Culfa, Ezgi Odabasi, Navin Rauniyar, John R Yates, Elif N Firat-Karalar
Centriolar satellites are membrane-less structures that localize and move around the centrosome/cilium complex in a microtubule-dependent manner. They play important roles in centrosome/cilium-related processes including protein trafficking to the centrosome/cilium complex and ciliogenesis, and are implicated in ciliopathies. Despite the important regulatory roles of centriolar satellites in the assembly and function of the centrosome/cilium complex, the molecular mechanism of their functions remains poorly understood...
February 24, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28230057/ensemble-and-single-molecule-dynamics-of-ift-dynein-in-caenorhabditis-elegans-cilia
#19
Jona Mijalkovic, Bram Prevo, Felix Oswald, Pierre Mangeol, Erwin J G Peterman
Cytoplasmic dyneins drive microtubule-based, minus-end directed transport in eukaryotic cells. Whereas cytoplasmic dynein 1 has been widely studied, IFT dynein has received far less attention. Here, we use fluorescence microscopy of labelled motors in living Caenorhabditis elegans to investigate IFT-dynein motility at the ensemble and single-molecule level. We find that while the kinesin composition of motor ensembles varies along the track, the amount of dynein remains relatively constant. Remarkably, this does not result in directionality changes of cargo along the track, as has been reported for other opposite-polarity, tug-of-war motility systems...
February 23, 2017: Nature Communications
https://www.readbyqxmd.com/read/28217833/lengthening-primary-cilia-enhances-cellular-mechanosensitivity
#20
M Spasic, C R Jacobs
The primary cilium is a mechanosensor in a variety of mammalian cell types, initiating and directing intracellular signalling cascades in response to external stimuli. When primary cilia formation is disrupted, cells have diminished mechanosensitivity and an abrogated response to mechanical stimulation. Due to this important role, we hypothesised that increasing primary cilia length would enhance the downstream response and therefore, mechanosensitivity. To test this hypothesis, we increased osteocyte primary cilia length with fenoldopam and lithium and found that cells with longer primary cilia were more mechanosensitive...
February 20, 2017: European Cells & Materials
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