Loss of Bardet-Biedl syndrome proteins alters the morphology and function of motile cilia in airway epithelia
about
BBS6, BBS10, and BBS12 form a complex with CCT/TRiC family chaperonins and mediate BBSome assemblyBardet-Biedl syndrome 3 (Bbs3) knockout mouse model reveals common BBS-associated phenotypes and Bbs3 unique phenotypesBardet-Biedl syndrome-associated small GTPase ARL6 (BBS3) functions at or near the ciliary gate and modulates Wnt signalingMotile cilia of human airway epithelia are chemosensoryDISC1 regulates primary cilia that display specific dopamine receptorsThe blind leading the obese: the molecular pathophysiology of a human obesity syndromeJoubert syndrome Arl13b functions at ciliary membranes and stabilizes protein transport in Caenorhabditis elegansSensory functions of motile cilia and implication for bronchiectasisEctopic expression of human BBS4 can rescue Bardet-Biedl syndrome phenotypes in Bbs4 null miceMouse models of ciliopathies: the state of the art.The BBSome controls IFT assembly and turnaround in cilia.CP110 and its network of partners coordinately regulate cilia assembly.Biology and therapy of inherited retinal degenerative disease: insights from mouse modelsNephrocystin proteins NPHP5 and Cep290 regulate BBSome integrity, ciliary trafficking and cargo deliveryTranscriptional program of ciliated epithelial cells reveals new cilium and centrosome components and links to human diseaseLoss of Bardet-Biedl syndrome protein-8 (BBS8) perturbs olfactory function, protein localization, and axon targetingChaperonin genes on the rise: new divergent classes and intense duplication in human and other vertebrate genomesThe future of ciliary and flagellar membrane research.The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagella.Heterotrimeric kinesin-II is necessary and sufficient to promote different stepwise assembly of morphologically distinct bipartite cilia in Drosophila antenna.CCTalpha and CCTdelta chaperonin subunits are essential and required for cilia assembly and maintenance in Tetrahymena.A role for central spindle proteins in cilia structure and function.New structural and functional contexts of the Dx[DN]xDG linear motif: insights into evolution of calcium-binding proteinsTemporal relationship between primary and motile ciliogenesis in airway epithelial cells.Ciliopathies: the trafficking connection.The retinitis pigmentosa protein RP2 interacts with polycystin 2 and regulates cilia-mediated vertebrate development.Accumulation of non-outer segment proteins in the outer segment underlies photoreceptor degeneration in Bardet-Biedl syndrome.Educational paper: ciliopathies.Paramecium BBS genes are key to presence of channels in Cilia.Cycling of the signaling protein phospholipase D through cilia requires the BBSome only for the export phase.Primary ciliary dyskinesia and associated sensory ciliopathiesEx vivo method for high resolution imaging of cilia motility in rodent airway epithelia.Pleiotropic genetic syndromes with developmental abnormalities associated with obesity.Next-generation sequence analysis of genes associated with obesity and nonalcoholic fatty liver disease-related cirrhosis in extreme obesityUnraveling the genetic basis of aspirin hypersensitivity in asthma beyond arachidonate pathways.Primary cilia in neurodevelopmental disorders.A missense mutation in Katnal1 underlies behavioural, neurological and ciliary anomalies.Chibby functions to preserve normal ciliary morphology through the regulation of intraflagellar transport in airway ciliated cells.Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus.Motile cilia defects in diseases other than primary ciliary dyskinesia: The contemporary diagnostic and research role for transmission electron microscopy.
P2860
4278a7addb94909c94d30e821e31b4c1c9598d314890e0c22224bc298ecb8486496ff1725a610c2a76c6ca2f70560d212f6ac753e1b9ace7b84e4f957fe41bda259199fec194f01ea1e47925a351a60181f429c72c892e13f959da7b8e96864ef21e616d8958c74d9bf991f5497ba288047225808f7394cfaafdbe26a936ad3231653b96602b61a0980bf35bb9208b3dd2695f60de132a28fbb5cf7dd7a2e702e5891b3549e7eb12bc1ef5359818cc697bd18ff5eefe6d306f10f91898562ab8def8e3403bda6910
P248
Q24295491-3B6FFEDF-6FF7-4F2D-873C-AADBD86DA0D1Q24298940-CBD50F3F-3404-4996-9DE5-C7BFEBDB867EQ24301800-EB56988E-6EFF-401F-9570-C98CBC83DCE7Q24317210-A21C4743-3512-4954-A6E6-794F4952A3ACQ24318393-27E924CF-E416-47E1-889E-A83434F56BCCQ24595694-D2A1049D-E0D3-4524-97D7-18E15C729C3DQ24614362-4BDDA0A3-D1F4-48CA-8EA7-4F1253E3BBB8Q26992107-5BB617F2-8E47-429A-A583-E7D7BE8F55B0Q27334338-3C1D4135-7B10-4B36-A267-381DF073165FQ27692039-F363369A-C599-4D73-A9D1-F26D6D6D5017Q28000109-D31F784D-734E-42C2-8B54-68349E599027Q28000138-6E0017F2-14CD-41E6-A446-F2C000F96403Q28084726-469248BF-B2DC-4036-A0C0-9DCFAF79882FQ28116651-8763F736-4B3F-4D4C-B250-BB7CD4F1D906Q28118826-F93DD0AF-8B21-4E96-8DFA-C466766C5BB8Q28591892-4C5B66A7-E7B1-4F41-ADE2-564CA517A752Q28748272-BCAD8334-F7BB-4C58-BC1E-2AAE0A492DB1Q30424945-104D657C-7326-4297-BD9B-69D70FC63D5AQ30435765-B540CB12-8495-43A0-A99C-3CFD447526FCQ30498706-3A8DECB4-20CA-4087-956E-44D1D54A6B5CQ33587559-9D429C78-3CB3-4667-925E-2B9DC4207186Q33874431-D1B4F261-4CF2-46C4-9B88-2E9E8A38E330Q33947979-13F2EEE5-9D68-4FA9-ABFD-BB90F587BF96Q34095893-EC7C4963-0CA8-4378-ADB4-A1EF01823549Q34207085-1ED9317D-AE79-4A2E-B9B8-3EBAD3C1BF6FQ34211989-014E9A84-D8F1-4E18-9B03-1AEE3F71A025Q35961452-F63A0FCF-2B7B-4346-80BB-89ABD33DC7CEQ36166406-CD8FB2A6-FC70-4CFA-9D2C-C97EBCE2F7DDQ36564940-CF295047-2090-45CA-8B07-66FAFFD67A7BQ36772553-2C11F2CC-AB8B-4CC9-99E5-9CD100B6D124Q36968964-D219D58B-0208-454B-9AD4-706E039693B1Q37371829-688CA064-3BB0-4F77-AC8E-B6BAC8E4C511Q37602156-C2539025-8CCD-4DCF-9B8C-811BF3AA053FQ37693953-4184F94F-DA36-4322-A726-619767FF8CC6Q38134073-4F09E861-0A7E-44AE-9BA6-64C91115CE27Q38168054-BB9B84B1-8A48-43EF-95C3-1A730B120D13Q38857397-E808938C-4184-4F31-AA86-59560B13122FQ39983088-7B1F60B9-9F5E-4D23-83D1-22EF272DF491Q40736117-C991DB2D-F877-42A1-8EFE-E4026BC63CFDQ41596398-935127B3-FBB2-424E-A6AC-4547350BA2E1
P2860
Loss of Bardet-Biedl syndrome proteins alters the morphology and function of motile cilia in airway epithelia
description
2008 nî lūn-bûn
@nan
2008 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի մարտին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@ast
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en-gb
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@nl
type
label
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@ast
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en-gb
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@nl
prefLabel
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@ast
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en-gb
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@nl
P2093
P2860
P921
P356
P1476
Loss of Bardet-Biedl syndrome ...... tile cilia in airway epithelia
@en
P2093
Alok S Shah
Charles C Searby
Darryl Nishimura
Kevin Bugge
Kim A Brogden
Michael J Welsh
Michael P Andrews
Sara L Farmen
Thomas O Moninger
Thomas R Businga
P2860
P304
P356
10.1073/PNAS.0712327105
P407
P577
2008-02-25T00:00:00Z