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10.1242/dmm.019083 http://scihub22266oqcxt.onion/10.1242/dmm.019083 C4457033!4457033 !26035863     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=26035863 &cmd=llinks): Failed to open stream: HTTP request failed! 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The Meckel-Gruber syndrome protein TMEM67 controls basal body positioning and epithelial branching morphogenesis in mice via the non-canonical Wnt pathway |pdf=|usr=}}{{26035863 }} gab.com Text The Meckel-Gruber syndrome protein TMEM67 controls basal body positioning and epithelial branching morphogenesis in mice via the non-canonical Wnt pathway JO: Dis Model Mech http://www.kidney.de/pget.php?&tw=1&pmid=26035863 #FOAvip Ciliopathies are a group of developmental disorders that manifest with multi-organ anomalies. Mutations in TMEM67 (MKS3) cause a range of human ciliopathies, including Meckel-Gruber and Joubert syndromes. In this study we describe multi-organ developmental abnormalities in the Tmem67(tm1Dgen/H1) knockout mouse that closely resemble those seen in Wnt5a and Ror2 knockout mice. These include pulmonary hypoplasia, ventricular septal defects, shortening of the body longitudinal axis, limb abnormalities, and cochlear hair cell stereociliary bundle orientation and basal body/kinocilium positioning defects. The basal body/kinocilium complex was often uncoupled from the hair bundle, suggesting aberrant basal body migration, although planar cell polarity and apical planar asymmetry in the organ of Corti were normal. TMEM67 (meckelin) is essential for phosphorylation of the non-canonical Wnt receptor ROR2 (receptor-tyrosine-kinase-like orphan receptor 2) upon stimulation with Wnt5a-conditioned medium. ROR2 also colocalises and interacts with TMEM67 at the ciliary transition zone. Additionally, the extracellular N-terminal domain of TMEM67 preferentially binds to Wnt5a in an in vitro binding assay. Cultured lungs of Tmem67 mutant mice failed to respond to stimulation of epithelial branching morphogenesis by Wnt5a. Wnt5a also inhibited both the Shh and canonical Wnt/?-catenin signalling pathways in wild-type embryonic lung. Pulmonary hypoplasia phenotypes, including loss of correct epithelial branching morphogenesis and cell polarity, were rescued by stimulating the non-canonical Wnt pathway downstream of the Wnt5a-TMEM67-ROR2 axis by activating RhoA. We propose that TMEM67 is a receptor that has a main role in non-canonical Wnt signalling, mediated by Wnt5a and ROR2, and normally represses Shh signalling. Downstream therapeutic targeting of the Wnt5a-TMEM67-ROR2 axis might, therefore, reduce or prevent pulmonary hypoplasia in ciliopathies and other congenital conditions. Twit Text FOAVip The Meckel-Gruber syndrome protein TMEM67 controls basal body positioning and epithelial branching morphogenesis in mice via the non-canonical Wnt pathway ????Dis Model Mech http://www.kidney.de/pget.php?&tw=1&pmid=26035863 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26035863 #FOAvip English Wikipedia <ref>{{Cite pmid|26035863 }}</ref> The Meckel-Gruber syndrome protein TMEM67 controls basal body positioning and epithelial branching morphogenesis in mice via the non-canonical Wnt pathway #MMPMID26035863 Abdelhamed ZA ; Natarajan S ; Wheway G ; Inglehearn CF ; Toomes C ; Johnson CA ; Jagger DJ Dis Model Mech 2015[Jun]; 8 (6 ): 527-41 PMID26035863 show ga Ciliopathies are a group of developmental disorders that manifest with multi-organ anomalies. Mutations in TMEM67 (MKS3) cause a range of human ciliopathies, including Meckel-Gruber and Joubert syndromes. In this study we describe multi-organ developmental abnormalities in the Tmem67(tm1Dgen/H1) knockout mouse that closely resemble those seen in Wnt5a and Ror2 knockout mice. These include pulmonary hypoplasia, ventricular septal defects, shortening of the body longitudinal axis, limb abnormalities, and cochlear hair cell stereociliary bundle orientation and basal body/kinocilium positioning defects. The basal body/kinocilium complex was often uncoupled from the hair bundle, suggesting aberrant basal body migration, although planar cell polarity and apical planar asymmetry in the organ of Corti were normal. TMEM67 (meckelin) is essential for phosphorylation of the non-canonical Wnt receptor ROR2 (receptor-tyrosine-kinase-like orphan receptor 2) upon stimulation with Wnt5a-conditioned medium. ROR2 also colocalises and interacts with TMEM67 at the ciliary transition zone. Additionally, the extracellular N-terminal domain of TMEM67 preferentially binds to Wnt5a in an in vitro binding assay. Cultured lungs of Tmem67 mutant mice failed to respond to stimulation of epithelial branching morphogenesis by Wnt5a. Wnt5a also inhibited both the Shh and canonical Wnt/?-catenin signalling pathways in wild-type embryonic lung. Pulmonary hypoplasia phenotypes, including loss of correct epithelial branching morphogenesis and cell polarity, were rescued by stimulating the non-canonical Wnt pathway downstream of the Wnt5a-TMEM67-ROR2 axis by activating RhoA. We propose that TMEM67 is a receptor that has a main role in non-canonical Wnt signalling, mediated by Wnt5a and ROR2, and normally represses Shh signalling. Downstream therapeutic targeting of the Wnt5a-TMEM67-ROR2 axis might, therefore, reduce or prevent pulmonary hypoplasia in ciliopathies and other congenital conditions. |*Body Patterning [MESH] |*Morphogenesis [MESH] |*Wnt Signaling Pathway [MESH] |Animals [MESH] |Animals, Newborn [MESH] |Cell Differentiation [MESH] |Cell Polarity [MESH] |Cilia/metabolism [MESH] |Ciliary Motility Disorders/*metabolism [MESH] |Embryo, Mammalian/abnormalities/metabolism [MESH] |Encephalocele/*metabolism [MESH] |Epithelium/*embryology/metabolism [MESH] |HEK293 Cells [MESH] |Humans [MESH] |Lung/embryology/metabolism [MESH] |Membrane Proteins/deficiency/*metabolism [MESH] |Mice [MESH] |Mutation/genetics [MESH] |Organ of Corti/abnormalities/embryology/pathology [MESH] |Phenotype [MESH] |Phosphorylation [MESH] |Polycystic Kidney Diseases/*metabolism [MESH] |Protein Binding [MESH] |Protein Structure, Tertiary [MESH] |Receptor Tyrosine Kinase-like Orphan Receptors/metabolism [MESH] |Retinitis Pigmentosa [MESH] |Stereocilia/metabolism [MESH] |Wnt Proteins/metabolism [MESH] |Wnt-5a Protein [MESH]The Meckel-Gruber syndrome protein TMEM67 controls basal body position(epmc).pdf DeepDyve Pubget Overpricing