Research Topics
Genomes and Genes | William A HortonSummaryAffiliation: Shriners Hospital for Children Country: USA Publications
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Publications
AchondroplasiaWilliam A Horton
Research Center, Shriners Hospital for Children and Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
Lancet 370:162-72. 2007..Possible future treatments include chemical inhibition of receptor signalling, antibody blockade of receptor activation, and alteration of pathways that modulate the downstream propagation of FGFR3 signals...- Crude subcellular fractionation of cultured mammalian cell linesPaul Holden
Research Center, Shriners Hospitals for Children, Portland, Oregon 97239, USA
BMC Res Notes 2:243. 2009..Currently published protocols involve time consuming centrifugation steps which may require expensive equipment and commercially available kits can be prohibitively expensive when handling large or multiple samples... - International workshop on the Skeletal Growth Plate Stevenson, Washington, June 11-15, 2006William A Horton
Research Center, Shriners Hospital for Children, Oregon Health and Science University, Portland, Oregon, United States
Matrix Biol 26:324-9. 2007 - Recent milestones in achondroplasia researchWilliam A Horton
Research Center, Shriners Hospital for Children, Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon 97221, USA
Am J Med Genet A 140:166-9. 2006 - FGFs in endochondral skeletal developmentWilliam A Horton
Research Center, Shriners Hospital for Children, and Molecular and Medical Genetics, Oregon Health and Sciences University, Portland, OR 97239, USA
Trends Endocrinol Metab 20:341-8. 2009..This review focuses on the roles of FGFs and FGFRs in endochondral skeletal development, with special attention given to concepts that have emerged in the past few years... - Skeletal development: insights from targeting the mouse genomeWilliam A Horton
Shriners Hospital for Children, Oregon Health and Science University, 3101 Sam Jackson Park Road, Portland, OR 97239 3009, USA
Lancet 362:560-9. 2003..Technical advances suggest that genetic engineering in mice will continue to be highly instructive in the field of skeletal biology... - The evolving definition of a chondrodysplasia?William A Horton
Research Center, Shriners Hospital, Department of Molecular and Medical Genetics, Oregon Health and Science University, 3101 SW Sam Jackson Park Road, Portland, OR 97201, USA
Pediatr Pathol Mol Med 22:47-52. 2003..Indeed, the way in which a specific chondrodysplasia is defined has evolved substantially over the past several decades. David Rimoin, to whom this special issue is dedicated, has played a vital role in this evolution... - Fibroblast growth factor receptor 3 mutations in achondroplasia and related forms of dwarfismWilliam A Horton
Research Center, Shriners Hospital for Children, Portland, OR, USA
Rev Endocr Metab Disord 3:381-5. 2002 - Ligand activation leads to regulated intramembrane proteolysis of fibroblast growth factor receptor 3Catherine R Degnin
Research Center, Shriners Hospital for Children, Portland, OR 97239, USA
Mol Biol Cell 22:3861-73. 2011..In this way we demonstrate a mechanism for the nuclear localization of FGFR3 in response to ligand activation, which may occur in both development and disease... - FGFs in endochondral skeletal developmentCatherine R Degnin
Shriners Hospital and Molecular and Medical Genetics and Cell and Developmental Biology, Oregon Health and Sciences University, Portland, Oregon 97239, USA
J Cell Biochem 110:1046-57. 2010..This prospect will focus on the current understanding of these events during endochondral skeletal development with special attention given to concepts that have emerged in the past few years... - Fibroblast growth factor receptor 3 (FGFR3) is a strong heat shock protein 90 (Hsp90) client: implications for therapeutic manipulationMelanie B Laederich
Research Center, Shriners Hospital for Children, Department of Cell and Developmental Biology, Oregon Health and Science University, Portland, Oregon 97239, USA
J Biol Chem 286:19597-604. 2011..Our results establish FGFR3 as a strong Hsp90 client and suggest that modulating Hsp90 chaperone complexes may beneficially influence the stability and function of FGFR3 in disease... - Confocal/TEM overlay microscopy: a simple method for correlating confocal and electron microscopy of cells expressing GFP/YFP fusion proteinsDouglas R Keene
Research Department, Shriners Hospital for Children, Portland, OR 97239, USA
Microsc Microanal 14:342-8. 2008..The method requires no specialized specimen preparation equipment; specimens are taken from live cultures to embedding within 8 h, and confocal transmission overlay microscopy can be completed within a few hours... - Y-position collagen II mutation disrupts cartilage formation and skeletal development in a transgenic mouse model of spondyloepiphyseal dysplasiaKelly G Gaiser
Research Center, Shriners Hospital for Children, Portland, Oregon 97201, USA
J Bone Miner Res 17:39-47. 2002.... - Secretion of cartilage oligomeric matrix protein is affected by the signal peptidePaul Holden
Research Center, Shriners Hospitals for Children, Portland, Oregon 97239, USA
J Biol Chem 280:17172-9. 2005..They also imply a previously unappreciated role for the signal peptide in the regulation of protein secretion beyond targeting to the endoplasmic reticulum... - Sprouty 2 disturbs FGFR3 degradation in thanatophoric dysplasia type II: a severe form of human achondroplasiaChangsheng Guo
Research Center, Shriners Hospital for Children, 3101 S W Sam Jackson Park Road, Portland, OR 97239, USA
Cell Signal 20:1471-7. 2008..They provide novel insight into the pathogenesis of this group of human skeletal dysplasias and identify a mechanism that potentially could be targeted therapeutically... - Defective lysosomal targeting of activated fibroblast growth factor receptor 3 in achondroplasiaJay Y Cho
Research Center, Shriners Hospital for Children, and Departmentof Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
Proc Natl Acad Sci U S A 101:609-14. 2004..The lysosomal targeting defect is additive to other mechanisms proposed to explain the pathogenesis of ACH... - Confocal fluorescence spectroscopy of subcutaneous cartilage expressing green fluorescent protein versus cutaneous collagen autofluorescenceDaniel S Gareau
Oregon Health and Science University, Department of Biomedical Engineering and Dermatology, Shriners Hospital for Crippled Children, Portland, Oregon 97239, USA
J Biomed Opt 9:254-8. 2004..13 and 0.48, respectively. The study demonstrates that a topical optical fiber measurement is affected by collagen autofluorescence, while a confocal microscope can detect subdermal fluorescence while rejecting collagen autofluorescence... - Achondroplasia: pathogenesis and implications for future treatmentMelanie B Laederich
Research Center, Shriners Hospital for Children, Department of Cell and Developmental Biology, Oregon Health and Science University, Portland, Oregon 97239, USA
Curr Opin Pediatr 22:516-23. 2010..Currently used preclinical models are discussed in the context of recent advances with a special focus on C-type natriuretic peptide... - Targeted expression of SHH affects chondrocyte differentiation, growth plate organization, and Sox9 expressionSara Tavella
Department of Oncology, Biology and Genetics, University of Genoa Medical School, Genoa, Italy
J Bone Miner Res 19:1678-88. 2004..Overexpression caused a lethal craniorachischisis with major alterations in long bones because of defects in chondrocyte differentiation... - Transient dimerization and interaction with ERGIC-53 occur in the fibroblast growth factor receptor 3 early secretory pathwayPatricia M J Lievens
Division of Biochemistry, Department of Morphological and Biomedical Sciences, University of Verona Medical School, Verona, Italy
Int J Biochem Cell Biol 40:2649-59. 2008..Furthermore, the TDII/ERGIC-53 complex formation may function as a checkpoint for FGFR3 sorting downstream the endoplasmic reticulum. These findings have implications for understanding the pathogenesis of FGFR3-related disorders... - BMP action in skeletogenesis involves attenuation of retinoid signalingLisa M Hoffman
Department of Physiology, Faculty of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada N6A 5C1
J Cell Biol 174:101-13. 2006..Collectively, these findings provide novel insights into BMP action and demonstrate that BMP signaling governs the fate of prechondrogenic mesenchyme, at least in part, through regulation of retinoid signaling... - Identification of STAT-1 as a molecular target of IGFBP-3 in the process of chondrogenesisAnna Spagnoli
Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee 37232 2579, USA
J Biol Chem 277:18860-7. 2002..We have demonstrated that STAT-1 is a major intracellular signaling and transcriptional target of the IGF-independent apoptotic effect of IGFBP-3 in chondrogenesis... - Developmental biology in orthopaedics. Summary of the 2006 AAOS research symposiumBenjamin A Alman
Division of Orthopaedic Surgery and Program in Developmental Biology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada
J Bone Joint Surg Am 89:668-71. 2007
Research Grants
- International Workshop on Skeletal GrowthWilliam Horton; Fiscal Year: 2006..It will be held at the Skamania Lodge near Portland, Oregon on June 11-15, 2006. The content of this Workshop has broad implications related to the missions of NICHD, NIAMS, and NIDCR. ..