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Roberto Mendoza-Londono Somayyeh Fahiminiya Jacek Majewski CareRare Canada Consortium Martine Tétreault Javad Nadaf Peter Kannu Etienne Sochett Andrew Howard Jennifer Stimec Lucie Dupuis Paul Roschger Klaus Klaushofer Telma Palomo Jean Ouellet Hadil Al-Jallad John?S. Mort Pierre Moffatt Sergei Boudko Hans-Peter B?chinger Frank Rauch 《American journal of human genetics》2015,96(6):979-985
Secreted protein, acidic, cysteine-rich (SPARC) is a glycoprotein that binds to collagen type I and other proteins in the extracellular matrix. Using whole-exome sequencing to identify the molecular defect in two unrelated girls with severe bone fragility and a clinical diagnosis of osteogenesis imperfecta type IV, we identified two homozygous variants in SPARC (GenBank: ; c.497G>A [p.Arg166His] in individual 1; c.787G>A [p.Glu263Lys] in individual 2). Published modeling and site-directed mutagenesis studies had previously shown that the residues substituted by these mutations form an intramolecular salt bridge in SPARC and are essential for the binding of SPARC to collagen type I. The amount of SPARC secreted by skin fibroblasts was reduced in individual 1 but appeared normal in individual 2. The migration of collagen type I alpha chains produced by these fibroblasts was mildly delayed on SDS-PAGE gel, suggesting some overmodification of collagen during triple helical formation. Pulse-chase experiments showed that collagen type I secretion was mildly delayed in skin fibroblasts from both individuals. Analysis of an iliac bone sample from individual 2 showed that trabecular bone was hypermineralized on the material level. In conclusion, these observations show that homozygous mutations in SPARC can give rise to severe bone fragility in humans. NM_003118.3相似文献
953.
Markus Schueler Daniela?A. Braun Gayathri Chandrasekar Heon?Yung Gee Timothy?D. Klasson Jan Halbritter Andrea Bieder Jonathan?D. Porath Rannar Airik Weibin Zhou Joseph?J. LoTurco Alicia Che Edgar?A. Otto Detlef B?ckenhauer Neil?J. Sebire Tomas Honzik Peter?C. Harris Sarah?J. Koon Meral Gunay-Aygun Sophie Saunier Klaus Zerres Nadina?Ortiz Bruechle Joost?P.H. Drenth Laurence Pelletier Isabel Tapia-Páez Richard?P. Lifton Rachel?H. Giles Juha Kere Friedhelm Hildebrandt 《American journal of human genetics》2015,96(1):81-92
Nephronophthisis-related ciliopathies (NPHP-RC) are recessive diseases characterized by renal dysplasia or degeneration. We here identify mutations of DCDC2 as causing a renal-hepatic ciliopathy. DCDC2 localizes to the ciliary axoneme and to mitotic spindle fibers in a cell-cycle-dependent manner. Knockdown of Dcdc2 in IMCD3 cells disrupts ciliogenesis, which is rescued by wild-type (WT) human DCDC2, but not by constructs that reflect human mutations. We show that DCDC2 interacts with DVL and DCDC2 overexpression inhibits β-catenin-dependent Wnt signaling in an effect additive to Wnt inhibitors. Mutations detected in human NPHP-RC lack these effects. A Wnt inhibitor likewise restores ciliogenesis in 3D IMCD3 cultures, emphasizing the importance of Wnt signaling for renal tubulogenesis. Knockdown of dcdc2 in zebrafish recapitulates NPHP-RC phenotypes, including renal cysts and hydrocephalus, which is rescued by a Wnt inhibitor and by WT, but not by mutant, DCDC2. We thus demonstrate a central role of Wnt signaling in the pathogenesis of NPHP-RC, suggesting an avenue for potential treatment of NPHP-RC. 相似文献
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Dynamic modelling of high biomass density cultivation and biohydrogen production in different scales of flat plate photobioreactors 下载免费PDF全文
Dongda Zhang Pongsathorn Dechatiwongse Ehecatl Antonio del Rio‐Chanona Geoffrey C. Maitland Klaus Hellgardt Vassilios S. Vassiliadis 《Biotechnology and bioengineering》2015,112(12):2429-2438
This paper investigates the scaling‐up of cyanobacterial biomass cultivation and biohydrogen production from laboratory to industrial scale. Two main aspects are investigated and presented, which to the best of our knowledge have never been addressed, namely the construction of an accurate dynamic model to simulate cyanobacterial photo‐heterotrophic growth and biohydrogen production and the prediction of the maximum biomass and hydrogen production in different scales of photobioreactors. To achieve the current goals, experimental data obtained from a laboratory experimental setup are fitted by a dynamic model. Based on the current model, two key original findings are made in this work. First, it is found that selecting low‐chlorophyll mutants is an efficient way to increase both biomass concentration and hydrogen production particularly in a large scale photobioreactor. Second, the current work proposes that the width of industrial scale photobioreactors should not exceed 0.20 m for biomass cultivation and 0.05 m for biohydrogen production, as severe light attenuation can be induced in the reactor beyond this threshold. Biotechnol. Bioeng. 2015;112: 2429–2438. © 2015 The Authors. Biotechnology and Bioengineering Published by Wiley Peiodicals, Inc. 相似文献
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Measuring the efficiency of fen restoration on carabid beetles and vascular plants: a case study from north‐eastern Germany 下载免费PDF全文
The aim of the study was to assess the effects of fen rewetting on carabid beetle and vascular plant assemblages within riverine fens along the river Peene in north‐eastern Germany. Drained (silage grassland), rewetted (restored formerly drained silage grassland), and near‐natural (fairly pristine) stands were compared. Eighty‐four beetle species (7,267 individuals) and 135 plant species were recorded. The richness of vascular plant species and the number of endangered species were highest on near‐natural fens. Fourteen years of rewetting did not increase plant species numbers compared with drained fens. For carabid beetles, however, species richness and the number of stenotopic species were highest on rewetted fens. Rewetting caused the replacement of generalist carabids by wetland specialists, but did not provide suitable habitat for specialist fen carabids or for plant species of oligo‐ or mesotrophic fen communities. Therefore, raising the water table on fens with nutrient‐rich, degraded peat was not sufficient for restoring species assemblages of intact fens, although water level was the most important environmental factor separating species assemblages. Our study illustrated that insects and plants may respond differentially to restoration, stressing the need to consider different taxa when assessing the efficiency of fen restoration. Furthermore, species assemblages of intact fens could not be restored within 14 years, highlighting the importance of conserving pristine habitat. 相似文献
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