SPRED1

Summary

Gene Symbol: SPRED1
Description: sprouty-related, EVH1 domain containing 1
Alias: NFLS, hSpred1, spred-1, sprouty-related, EVH1 domain-containing protein 1, suppressor of Ras/MAPK activation
Species: human

Top Publications

  1. ncbi Germline loss-of-function mutations in SPRED1 cause a neurofibromatosis 1-like phenotype
    Hilde Brems
    Department of Human Genetics, Catholic University Leuven, 3000 Leuven, Belgium
    Nat Genet 39:1120-6. 2007
  2. doi SPRED1 mutations (Legius syndrome): another clinically useful genotype for dissecting the neurofibromatosis type 1 phenotype
    G Spurlock
    Institute of Medical Genetics, Cardiff University, Cardiff, UK
    J Med Genet 46:431-7. 2009
  3. ncbi The Sprouty-related protein, Spred-1, localizes in a lipid raft/caveola and inhibits ERK activation in collaboration with caveolin-1
    Atsushi Nonami
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, Higashi ku, Fukuoka 812 8582, Japan
    Genes Cells 10:887-95. 2005
  4. doi Clinical and mutational spectrum of neurofibromatosis type 1-like syndrome
    Ludwine Messiaen
    Medical Genomics Laboratory, Department of Genetics, University of Alabama at Birmingham, 720 20th St S, Birmingham, AL 35294, USA
    JAMA 302:2111-8. 2009
  5. doi SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotype
    E Pasmant
    UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
    J Med Genet 46:425-30. 2009
  6. ncbi Spred-1 negatively regulates interleukin-3-mediated ERK/mitogen-activated protein (MAP) kinase activation in hematopoietic cells
    Atsushi Nonami
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    J Biol Chem 279:52543-51. 2004
  7. pmc SPRED 1 mutations in a neurofibromatosis clinic
    Talia M Muram-Zborovski
    Department of Pathology, University of Utah, Salt Lake City, Utah, USA
    J Child Neurol 25:1203-9. 2010
  8. ncbi Spred is a Sprouty-related suppressor of Ras signalling
    T Wakioka
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Nature 412:647-51. 2001
  9. ncbi Molecular cloning of mammalian Spred-3 which suppresses tyrosine kinase-mediated Erk activation
    Reiko Kato
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Biochem Biophys Res Commun 302:767-72. 2003
  10. ncbi Legius Syndrome, an Update.Molecular Pathology of Mutations in SPRED1
    Hilde Brems
    Department of Human Genetics, KU Leuven, Leuven, Belgium
    Keio J Med 62:107-12. 2013

Research Grants

  1. Molecular Function of the Nf2 Tumor Suppressor, Merlin
    Andrea McClatchey; Fiscal Year: 2009
  2. Molecular Function of the Nf2 Tumor Suppressor, Merlin
    Andrea I McClatchey; Fiscal Year: 2010
  3. Comprehensive NF1 Mutational Analysis in Leukemia Cells
    Ludwine Messiaen; Fiscal Year: 2006

Scientific Experts

  • Talia M Muram-Zborovski
  • Olivier Dereure
  • Nadia Chuzhanova
  • Yvonne E Chiu
  • Taku Miyagawa
  • Cathy A Stevens
  • Santokh S Gill
  • Andrea McClatchey
  • LUDWINE MARIA MESSIAEN
  • Peter Lock
  • Katherine A Lane
  • Eric Pasmant
  • Hilde Brems
  • Akihiko Yoshimura
  • Eric Legius
  • Brigitte Widemann
  • Ellen Denayer
  • Reiko Kato
  • Atsushi Nonami
  • Koji Taniguchi
  • Takuma Ishizaki
  • Permeen Yusoff
  • Graeme R Guy
  • Sumana Chandramouli
  • Yan Zhang
  • Xing Guo Wang
  • Kathryn Zimmermann
  • Alain van Mil
  • Stephen E Hamby
  • Xin Ye
  • Seik Soon Khor
  • Felix Jansen
  • N Kachroo
  • Talia M Muram
  • Meena Upadhyaya
  • Irma B Stowe
  • Tong Zhou
  • Seema Singh
  • Rick van Minkelen
  • Kathrine B Sylvestersen
  • Mustapha Amyere
  • Emily Spencer
  • Takaharu Taketomi
  • Carlota A García-Domínguez
  • Lei Zhuang
  • Martina Quintanar-Audelo
  • Jin Bok Hwang
  • Stacy Visser
  • Timothy N Phoenix
  • Timothy Pepini
  • Dan Li
  • Magdalena Chmara
  • Anna Maja Nyström
  • Wojciech Lisik
  • Razieh Soltani-Arabshahi
  • Maria Giovanna di Bari
  • G Spurlock
  • Patricia Reant
  • Jason E Fish
  • Cindy Johne
  • Kazuko Saeki
  • Majid Momeny
  • Frank Kuhnert
  • Luca Vignatelli
  • Guo Hua Zhao
  • Yuriko Katoh
  • T Yoshida
  • Nicholas J Harmer
  • James A J King
  • Mine Harada
  • Kanta Miyoshi
  • Sarah Trimpin
  • A Yoshimura
  • Toru Wakioka
  • Corina H Metz
  • Pablo Reviriego
  • Paul J Hanson
  • Narumol Jariyasopit
  • Arianna Cattelan
  • Katsushi Tokunaga
  • Dao Qing Gong
  • Roger Atkinson
  • David H Viskochil
  • Tsukasa Sasaki
  • Sarah Vosen
  • Hui Juan Wang
  • Yan Li
  • Emmanuel Mignot
  • Sarah Watts-Justice
  • Rui Zhang

Detail Information

Publications67

  1. ncbi Germline loss-of-function mutations in SPRED1 cause a neurofibromatosis 1-like phenotype
    Hilde Brems
    Department of Human Genetics, Catholic University Leuven, 3000 Leuven, Belgium
    Nat Genet 39:1120-6. 2007
    We report germline loss-of-function mutations in SPRED1 in a newly identified autosomal dominant human disorder...
  2. doi SPRED1 mutations (Legius syndrome): another clinically useful genotype for dissecting the neurofibromatosis type 1 phenotype
    G Spurlock
    Institute of Medical Genetics, Cardiff University, Cardiff, UK
    J Med Genet 46:431-7. 2009
    Mutations of the SPRED1 gene, one of a family of Sprouty (Spry)/Spred proteins known to "downregulate" mitogen activated protein kinase (MAPK) signalling, have been identified in patients with a mild neurofibromatosis type 1 (NF1) ..
  3. ncbi The Sprouty-related protein, Spred-1, localizes in a lipid raft/caveola and inhibits ERK activation in collaboration with caveolin-1
    Atsushi Nonami
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, Higashi ku, Fukuoka 812 8582, Japan
    Genes Cells 10:887-95. 2005
    ..Furthermore, forced expression of exogenous Spred-1 in Cav-1-expressing cells further suppressed proliferation and ERK activation. These data suggest that Spred-1 inhibits ERK activation in collaboration with Cav-1...
  4. doi Clinical and mutational spectrum of neurofibromatosis type 1-like syndrome
    Ludwine Messiaen
    Medical Genomics Laboratory, Department of Genetics, University of Alabama at Birmingham, 720 20th St S, Birmingham, AL 35294, USA
    JAMA 302:2111-8. 2009
    Autosomal dominant inactivating sprouty-related EVH1 domain-containing protein 1 (SPRED1) mutations have recently been described in individuals presenting mainly with café au lait macules (CALMs), axillary freckling, and macrocephaly...
  5. doi SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotype
    E Pasmant
    UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
    J Med Genet 46:425-30. 2009
    Germline loss-of-function mutations in the SPRED1 gene have recently been identified in patients fulfilling the National Institutes of Health (NIH) diagnostic criteria for neurofibromatosis type 1 (NF1) but with no NF1 (neurofibromin 1) ..
  6. ncbi Spred-1 negatively regulates interleukin-3-mediated ERK/mitogen-activated protein (MAP) kinase activation in hematopoietic cells
    Atsushi Nonami
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    J Biol Chem 279:52543-51. 2004
    ..These data suggest that Spred-1 negatively regulates hematopoiesis by suppressing not only SCF-induced but also IL-3-induced ERK activation...
  7. pmc SPRED 1 mutations in a neurofibromatosis clinic
    Talia M Muram-Zborovski
    Department of Pathology, University of Utah, Salt Lake City, Utah, USA
    J Child Neurol 25:1203-9. 2010
    Legius syndrome, caused by SPRED1 mutations, has phenotypic overlap with neurofibromatosis type 1 (NF1) without tumorigenic manifestations...
  8. ncbi Spred is a Sprouty-related suppressor of Ras signalling
    T Wakioka
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Nature 412:647-51. 2001
    ..Instead, Spred inhibited the activation of MAP kinase by suppressing phosphorylation and activation of Raf. Spred may represent a class of proteins that modulate Ras-Raf interaction and MAP kinase signalling...
  9. ncbi Molecular cloning of mammalian Spred-3 which suppresses tyrosine kinase-mediated Erk activation
    Reiko Kato
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Biochem Biophys Res Commun 302:767-72. 2003
    ..The finding of Spred-3 revealed the presence of a novel family of regulators for the Ras/MAP kinase pathway, each member of which may have different specificities for extracellular signals...
  10. ncbi Legius Syndrome, an Update.Molecular Pathology of Mutations in SPRED1
    Hilde Brems
    Department of Human Genetics, KU Leuven, Leuven, Belgium
    Keio J Med 62:107-12. 2013
    ..In 2007 we reported that some individuals with multiple CALMs have a heterozygous mutation in the SPRED1 gene and have NF1-like syndrome, or Legius syndrome...
  11. doi Endothelial microparticle-mediated transfer of MicroRNA-126 promotes vascular endothelial cell repair via SPRED1 and is abrogated in glucose-damaged endothelial microparticles
    Felix Jansen
    Department of Internal Medicine II, University Hospital Bonn, Rheinische Friedrich Wilhelms University, Bonn, Germany F J, M H, A C, T S, S P, G N, N W Feinberg Cardiovascular Research Institute, Northwestern University School of Medicine, Chicago, IL X Y Institute of Physiology, University Hospital Bonn, Rheinische Friedrich Wilhelms University, Bonn, Germany D W, S V, B K F and Institute of Innate Immunity, Rheinische Friedrich Wilhelms University, Bonn, Germany B S F
    Circulation 128:2026-38. 2013
    ..The underlying mechanisms of endothelial cell repair are largely unknown. We sought to investigate whether endothelial microparticles (EMPs), released from apoptotic endothelial cells (ECs), influence EC repair...
  12. doi [Germinal loss-of-function mutations in the spred1 gene cause conditions similar to neurofibromatosis type 1]
    O Dereure
    Service de Dermatologie, Hopital Saint Eloi, 80, avenue Augustin Fliche, 34295 Montpellier Cedex 5, France
    Ann Dermatol Venereol 135:529-30. 2008
  13. doi MiR-126 promotes coxsackievirus replication by mediating cross-talk of ERK1/2 and Wnt/β-catenin signal pathways
    Xin Ye
    Department of Pathology and Laboratory Medicine, The Institute for Heart and Lung Health, St Paul s Hospital, University of British Columbia, Rm 166, 1081 Burrard Street, Vancouver, BC, V6Z1Y6, Canada
    Cell Mol Life Sci 70:4631-44. 2013
    ..and inhibitors, we proved that the upregulated miR-126 suppressed sprouty-related, EVH1 domain containing 1 (SPRED1) and in turn enhanced ERK1/2 activation...
  14. pmc Genome-wide association study of HLA-DQB1*06:02 negative essential hypersomnia
    Seik Soon Khor
    Department of Human Genetics, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
    Peerj 1:e66. 2013
    ..02E-07; NCKAP5), rs11854769 (P = 6.69E-07; SPRED1), and rs10988217 (P = 3...
  15. doi MicroRNA-126 expression is decreased in cultured primary chicken hepatocytes and targets the sprouty-related EVH1 domain containing 1 mRNA
    Xing Guo Wang
    College of Animal Science and Technology, Yangzhou University, Yangzhou, People s Republic of China
    Poult Sci 92:1888-96. 2013
    ..tissues, and it is believed to play a role in angiogenesis by repressing sprouty-related EVH1 domain containing 1 (Spred1)...
  16. doi Formation of nitro-PAHs from the heterogeneous reaction of ambient particle-bound PAHs with N2O5/NO3/NO2
    Kathryn Zimmermann
    Air Pollution Research Center, University of California, Riverside, California 92521, United States
    Environ Sci Technol 47:8434-42. 2013
    ..of particle-bound PAHs and formation of molecular weight (mw) 247 nitropyrenes (NPYs) and nitrofluoranthenes (NFLs), mw 273 nitrotriphenylenes (NTPs), nitrobenz[a]anthracenes (NBaAs), nitrochrysene (NCHR), and mw 297 nitrobenzo[a]..
  17. doi MicroRNA-1 enhances the angiogenic differentiation of human cardiomyocyte progenitor cells
    Alain van Mil
    Department of Cardiology, Division of Heart and Lungs, University Medical Center Utrecht, Heidelberglaan 100, Room G02 523, 3584 CX, Utrecht, The Netherlands
    J Mol Med (Berl) 91:1001-12. 2013
    ..miR-1 was found to directly target and inhibit the expression of sprouty-related EVH1 domain-containing protein 1 (Spred1). Knocking down Spred1 phenocopies the functional effect seen for miR-1 upregulation...
  18. pmc Screening in silico predicted remotely acting NF1 gene regulatory elements for mutations in patients with neurofibromatosis type 1
    Stephen E Hamby
    School of Science and Technology, Nottingham Trent University, Clifton Lane, Nottingham NG11 8NS, UK
    Hum Genomics 7:18. 2013
    ..These regions were then sequenced in 47 NF1 patients in whom no mutations had been found in either the NF1 or SPRED1 gene regions. Five patients were found to harbour DNA sequence variants in the distal H3K27ac-enriched region...
  19. doi [Relationship between Spred1 and acute myeloid leukemia]
    Yan Zhang
    Deprtment of Hematology, The First Affiliated Hospilal, China Medical University, Shenyang 110001, Liaoning Province, China
    Zhongguo Shi Yan Xue Ye Xue Za Zhi 21:1083-7. 2013
    b>SPRED1 protein coded by SPRED1 gene, a kind of tumors suppressor,belongs to Sprouty related protein family and mainly distributes in human brain...
  20. doi Café-au-lait macules and intertriginous freckling in piebaldism: clinical overlap with neurofibromatosis type 1 and Legius syndrome
    Cathy A Stevens
    Department of Pediatrics, University of Tennessee College of Medicine, Chattanooga, Tennessee, USA
    Am J Med Genet A 158:1195-9. 2012
    ..affected with piebaldism; the proband meeting diagnostic criteria for NF1 also underwent comprehensive NF1 and SPRED1 testing with no mutations detected...
  21. pmc A shared molecular mechanism underlies the human rasopathies Legius syndrome and Neurofibromatosis-1
    Irma B Stowe
    Helen Diller Family Comprehensive Cancer Center, University of California at San Francisco, San Francisco, California 94158, USA
    Genes Dev 26:1421-6. 2012
    ..Loss-of-function mutations in one feedback regulator of Ras/MAPK signaling, SPRED1 (Sprouty-related protein with an EVH1 domain), cause Legius syndrome, an autosomal dominant human disorder that ..
  22. pmc SPRED proteins provide a NF-ty link to Ras suppression
    Andrea I McClatchey
    Massachusetts General Hospital Center for Cancer Research, Department of Pathology, Harvard Medical School, Charlestown, Massachusetts 02129, USA
    Genes Dev 26:1515-9. 2012
    Mutations in the SPRED1 (Sprouty-related protein with an EVH [Ena/Vasp homology] domain 1) and NF1 (neurofibromatosis 1) genes underlie clinically related human disorders...
  23. pmc Defining an EPOR- regulated transcriptome for primary progenitors, including Tnfr-sf13c as a novel mediator of EPO- dependent erythroblast formation
    Seema Singh
    Center of Excellence in Stem Cell Biology and Regenerative Medicine Maine Medical Center Research Institute, Scarborough, Maine, United States of America
    PLoS ONE 7:e38530. 2012
    ..For example, not only Socs3 plus Socs2 but also Spred2, Spred1 and Eaf1 were EPO-induced as negative-feedback components...
  24. doi A cost savings approach to SPRED1 mutational analysis in individuals at risk for neurofibromatosis type 1
    Talia M Muram
    Department of Pathology, University of Utah, Utah, USA
    Am J Med Genet A 161:467-72. 2013
    ..Two genetic testing algorithms (SPRED1 testing alone, and NF1 mutation analysis with reflex to SPRED1) were compared against a baseline of no genetic ..
  25. pmc Evidence for downregulation of the negative regulator SPRED2 in clinical prostate cancer
    N Kachroo
    Translational Prostate Cancer Group, Hutchison MRC Research Centre, University of Cambridge, Cambridge CB1 0XZ, UK
    Br J Cancer 108:597-601. 2013
    b>SPRED1 and 2 are key negative regulators of MAPK signalling in mammalian cells. Here, we investigate the expression and functional role of SPREDs in prostate cancer.
  26. doi Association of Piebaldism, multiple café-au-lait macules, and intertriginous freckling: clinical evidence of a common pathway between KIT and sprouty-related, ena/vasodilator-stimulated phosphoprotein homology-1 domain containing protein 1 (SPRED1)
    Yvonne E Chiu
    Division of Pediatric Dermatology, Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA
    Pediatr Dermatol 30:379-82. 2013
    ..caused by Sprouty-related, Ena/vasodilator-stimulated phosphoprotein homology-1 domain containing protein 1 (SPRED1) mutations that also has multiple café-au-lait macules and intertriginous freckling...
  27. pmc Peripheral blood gene expression as a novel genomic biomarker in complicated sarcoidosis
    Tong Zhou
    Institute for Personalized Respiratory Medicine, The University of Illinois at Chicago, Chicago, Illinois, United States of America
    PLoS ONE 7:e44818. 2012
    ..TSHZ2; T cell/JAK-STAT pathway genes such as AKT3, CBLB, DLG1, IFNG, IL2RA, IL7R, ITK, JUN, MALT1, NFATC2, PLCG1, SPRED1)...
  28. doi Effects of environmentally relevant mixtures of persistent organic pollutants on the developmental neurobiology in rats
    Santokh Gill
    Toxicology Research Division, Health Products and Foods Branch, Health Canada, Ottawa, Ontario, Canada
    Toxicol Pathol 41:38-47. 2013
    ..In addition, structural genes including neurofilaments (NFLs) and microtubule-associated protein (MAP-2) were downregulated at least 2-fold or greater...
  29. doi Review and update of SPRED1 mutations causing Legius syndrome
    Hilde Brems
    Department of Human Genetics, Catholic University Leuven, Leuven, Belgium
    Hum Mutat 33:1538-46. 2012
    ..Legius syndrome is caused by germline loss-of-function SPRED1 mutations, resulting in overactivation of the RAS-MAPK signal transduction cascade...
  30. pmc What is the 'objective' differential factor of diarrhea in infancy?: Normal state versus diarrheal illness in infants with chronic frequent and loose stool
    Jin Bok Hwang
    Department of Pediatrics, Keimyung University, School of Medicine, Daegu, Korea
    Korean J Pediatr 53:1006-11. 2010
    This study aimed to identify 'objective' differential factors for normal frequent loose stool (NFLS) and diarrheal illness with dehydration and nutritional deficiency (DIDN) among infants with chronic frequent loose stool (CFLS).
  31. pmc Sprouty2 and Spred1-2 proteins inhibit the activation of the ERK pathway elicited by cyclopentenone prostanoids
    Carlota A García-Domínguez
    Unidad de Biologia Celular, Área de Biología Celular y del Desarrollo, Centro Nacional de Microbiologia, Instituto de Salud Carlos III ISCIII, Majadahonda, Madrid, Spain
    PLoS ONE 6:e16787. 2011
    ..We therefore found that ectopic overexpression in HeLa cells of human Sprouty2, or human Spred1 or 2, inhibits ERK1/2 and Elk-1 activation triggered by the cyclopentenone prostanoids PGA(1) and 15d-PGJ(2)...
  32. doi KITLG mutations cause familial progressive hyper- and hypopigmentation
    Mustapha Amyere
    Laboratory of Human Molecular Genetics, de Duve Institute, Universite Catholique de Louvain, Brussels, Belgium
    J Invest Dermatol 131:1234-9. 2011
    ..the Legius syndrome, characterized by familial café-au-lait spots and skin fold freckling, caused by mutations in SPRED1. We performed a genome-wide linkage analysis in seven families with FPHH, and identified linkage on 12q21...
  33. pmc Sprouty-related Ena/vasodilator-stimulated phosphoprotein homology 1-domain-containing protein (SPRED1), a tyrosine-protein phosphatase non-receptor type 11 (SHP2) substrate in the Ras/extracellular signal-regulated kinase (ERK) pathway
    Martina Quintanar-Audelo
    Institute of Molecular and Cell Biology, Signal Transduction Laboratory, 61 Biopolis Drive, Proteos 138673, Singapore
    J Biol Chem 286:23102-12. 2011
    ..Finally, we found two tyrosine residues on SPRED1 that are required, when phosphorylated, to inhibit Ras/ERK activation and identified Tyr-420 as a specific ..
  34. doi Identification of SPRED1 deletions using RT-PCR, multiplex ligation-dependent probe amplification and quantitative PCR
    Emily Spencer
    University of Alabama at Birmingham, Dept of Genetics, 35294 0024, USA
    Am J Med Genet A 155:1352-9. 2011
    Legius syndrome, is a recently identified autosomal dominant disorder caused by loss of function mutations in the SPRED1 gene, with individuals mainly presenting with multiple café-au-lait macules (CALM), freckling and macrocephaly...
  35. doi Interaction of the receptor FGFRL1 with the negative regulator Spred1
    Lei Zhuang
    Department of Clinical Research, University of Bern, 3010 Bern, Switzerland
    Cell Signal 23:1496-504. 2011
    ..we used the yeast two-hybrid system to demonstrate that FGFRL1 binds with its C-terminal, histidine-rich domain to Spred1 and to other proteins of the Sprouty/Spred family...
  36. doi miR126 positively regulates mast cell proliferation and cytokine production through suppressing Spred1
    Takuma Ishizaki
    Department of Microbiology and Immunology, Keio University School of Medicine, Shinjuku ku, Tokyo, Japan
    Genes Cells 16:803-14. 2011
    The protein known as Spred1 (Sprouty-related Ena/VASP homology-1 domain-containing protein) has been identified as a negative regulator of growth factor-induced ERK/mitogen-activated protein kinase activation...
  37. ncbi FGF signaling inhibitor, SPRY4, is evolutionarily conserved target of WNT signaling pathway in progenitor cells
    Yuriko Katoh
    M and M Medical BioInformatics, Hongo, Japan
    Int J Mol Med 17:529-32. 2006
    ..Here, transcriptional regulation of SPRY1, SPRY2, SPRY3, SPRY4, SPRED1, SPRED2, and SPRED3 genes by WNT/beta-catenin signaling cascade was investigated by using bioinformatics and human ..
  38. pmc Legius syndrome in fourteen families
    Ellen Denayer
    Department of Human Genetics, Catholic University of Leuven, Belgium
    Hum Mutat 32:E1985-98. 2011
    ..It is caused by germline loss-of-function SPRED1 mutations and is a member of the RAS-MAPK pathway syndromes...
  39. pmc miR-126 regulates angiogenic signaling and vascular integrity
    Jason E Fish
    Gladstone Institute of Cardiovascular Disease, San Francisco, CA 94158, USA
    Dev Cell 15:272-84. 2008
    ..in part by directly repressing negative regulators of the VEGF pathway, including the Sprouty-related protein SPRED1 and phosphoinositol-3 kinase regulatory subunit 2 (PIK3R2/p85-beta)...
  40. doi Effects of silibinin on cell growth and invasive properties of a human hepatocellular carcinoma cell line, HepG-2, through inhibition of extracellular signal-regulated kinase 1/2 phosphorylation
    Majid Momeny
    Department of Medical Genetics, University of Tehran, Tehran, Iran
    Eur J Pharmacol 591:13-20. 2008
    ..In addition, cell growth and proliferation may be inhibited by silibinin through down-regulation of Hec1...
  41. pmc Spred1 and TESK1--two new interaction partners of the kinase MARKK/TAO1 that link the microtubule and actin cytoskeleton
    Cindy Johne
    Max Planck Unit for Structural Molecular Biology, D 22607 Hamburg, Germany
    Mol Biol Cell 19:1391-403. 2008
    ..and identified two new interaction partners of MARKK, the regulatory Sprouty-related protein with EVH-1 domain1 (Spred1) and the testis-specific protein kinase (TESK1)...
  42. doi Experimental validation of circumferential, longitudinal, and radial 2-dimensional strain during dobutamine stress echocardiography in ischemic conditions
    Patricia Reant
    INSERM U828 and IFR4, University of Bordeaux 2, Bordeaux, France
    J Am Coll Cardiol 51:149-57. 2008
    ..The aim of this study was to assess and validate 2-dimensional (2D) strain for the detection of ischemia during dobutamine stress echocardiography (DSE)...
  43. ncbi [Gene screening in five Chinese families with hereditary spastic paraplegia with thin corpus callosum]
    Guo Hua Zhao
    Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, 410008 PR China
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 24:677-80. 2007
    ..To screen all ten genes between D15S971 and D15S1012 in five Chinese families with hereditary spastic paraplegia with thin corpus callosum (HSP-TCC)...
  44. ncbi Tesk1 interacts with Spry2 to abrogate its inhibition of ERK phosphorylation downstream of receptor tyrosine kinase signaling
    Sumana Chandramouli
    Institute of Molecular and Cell Biology, 61 Biopolis Drive, Proteos, Singapore 138673
    J Biol Chem 283:1679-91. 2008
    ..By selectively modulating the downstream effects of Spry2, Tesk1 may thus serve as a molecular determinant of the signaling outcome...
  45. ncbi Interobserver reliability of video recording in the diagnosis of nocturnal frontal lobe seizures
    Luca Vignatelli
    Department of Neurological Sciences, University of Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy
    Epilepsia 48:1506-11. 2007
    Nocturnal frontal lobe seizures (NFLS) show one or all of the following semeiological patterns: (1) paroxysmal arousals (PA: brief and sudden recurrent motor paroxysmal behavior); (2) hyperkinetic seizures (HS: motor attacks with complex ..
  46. ncbi Spred-2 steady-state levels are regulated by phosphorylation and Cbl-mediated ubiquitination
    Peter Lock
    Cell Signaling Laboratory, Department of Surgery, University of Melbourne, Royal Melbourne Hospital, Parkville 3050, Australia
    Biochem Biophys Res Commun 351:1018-23. 2006
    ..These results suggest a role for Spred-2 tyrosine phosphorylation and ubiquitination in controlling Spred-2 expression levels...
  47. ncbi Spreds, inhibitors of the Ras/ERK signal transduction, are dysregulated in human hepatocellular carcinoma and linked to the malignant phenotype of tumors
    T Yoshida
    Department of Medicine, Kurume University School of Medicine, Liver Cancer Division, Research Center for Innovative Cancer Therapy, and Center of the 21st Century COE Program for Medical Science, Kurume, Japan
    Oncogene 25:6056-66. 2006
    ..These data indicate that the reduction of Spred expression in HCC is one of the causes of the acquisition of malignant features. Thus, Spred could be not only a novel prognostic factor but also a new therapeutic target for human HCC...
  48. ncbi 1.15 A crystal structure of the X. tropicalis Spred1 EVH1 domain suggests a fourth distinct peptide-binding mechanism within the EVH1 family
    Nicholas J Harmer
    Department of Biochemistry, 80 Tennis Court Road, Cambridge, CB2 1GA, UK
    FEBS Lett 579:1161-6. 2005
    ..structure of the Enabled/vasodilator-stimulated phosphoprotein homology-1 (EVH1) domain from Xenopus tropicalis Spred1, solved to 1.15 A resolution...
  49. pmc Distinct requirements for the Sprouty domain for functional activity of Spred proteins
    James A J King
    Department of Surgery, 5th Floor Clinical Sciences Building, University of Melbourne, Royal Melbourne Hospital, Parkville, VIC 3050, Australia
    Biochem J 388:445-54. 2005
    ....
  50. ncbi The Sprouty-related protein, Spred, inhibits cell motility, metastasis, and Rho-mediated actin reorganization
    Kanta Miyoshi
    Division of Molecular and Cellular Immunology, Medical Institute of Bioregulation, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Oncogene 23:5567-76. 2004
    ..Using stably highly metastatic LM8 cells infected with the Spred1-Sendai virus vector, we demonstrated that Spred1 inhibits the metastasis of LM8 cells in nude mice...
  51. doi Attribution of vascular phenotypes of the murine Egfl7 locus to the microRNA miR-126
    Frank Kuhnert
    Division of Hematology, Department of Medicine, Stanford University School of Medicine, CCSR 1155, 269 Campus Drive, Stanford, CA 94305, USA
    Development 135:3989-93. 2008
    ..inhibited VEGF-dependent Akt and Erk signaling by derepression of the p85beta subunit of PI3 kinase and of Spred1, respectively...
  52. doi Spred1 is required for synaptic plasticity and hippocampus-dependent learning
    Ellen Denayer
    Department of Human Genetics and Laboratory of Biological Psychology, University of Leuven, B 3000 Leuven, Belgium
    J Neurosci 28:14443-9. 2008
    Germline mutations in SPRED1, a negative regulator of Ras, have been described in a neurofibromatosis type 1 (NF1)-like syndrome (NFLS) that included learning difficulties in some affected individuals...
  53. doi Fgf9 signalling stimulates Spred and Sprouty expression in embryonic mouse pancreas mesenchyme
    Kathrine B Sylvestersen
    Hagedorn Research Institute, Department of Developmental Biology, Gentofte, Denmark
    Gene Expr Patterns 11:105-11. 2011
    ..that several members of the Spred and Sprouty families are expressed in embryonic mouse pancreas and find Spred1 and -2 as well as Spry2 and -4 to be predominantly expressed in pancreatic mesenchyme...
  54. pmc Andes virus regulation of cellular microRNAs contributes to hantavirus-induced endothelial cell permeability
    Timothy Pepini
    Department of Molecular Genetics and Microbiology, Stony Brook University, Stony Brook, NY 11794 5222, USA
    J Virol 84:11929-36. 2010
    ..We further analyzed changes in miR-126, an EC-specific miRNA that regulates vascular integrity by suppressing SPRED1 and PIK3R2 mRNAs...
  55. pmc Direct association of Sprouty-related protein with an EVH1 domain (SPRED) 1 or SPRED2 with DYRK1A modifies substrate/kinase interactions
    Dan Li
    Institute of Molecular and Cell Biology, Signal Transduction Laboratory, 61 Biopolis Drive, Proteos 138673, Singapore
    J Biol Chem 285:35374-85. 2010
    The mammalian SPRED (Sprouty-related protein with an EVH1 domain) proteins include a family of three members, SPRED1-3. Currently, little is known about their biochemistry...
  56. pmc NF1 exon 22 analysis of individuals with the clinical diagnosis of neurofibromatosis type 1
    Talia M Muram-Zborovski
    Department of Pathology, University of Utah, SLC, Utah, USA
    Am J Med Genet A 152:1973-8. 2010
    ..b>SPRED1 mutations cause Legius syndrome, which clinically overlaps with this attenuated NF1 phenotype...
  57. pmc Spred1, a negative regulator of Ras-MAPK-ERK, is enriched in CNS germinal zones, dampens NSC proliferation, and maintains ventricular zone structure
    Timothy N Phoenix
    New York Neural Stem Cell Institute, Rensselaer, New York 12144, USA
    Genes Dev 24:45-56. 2010
    ..Sprouty-related protein with an EVH1 domain (Spred1) is a recently discovered negative Ras-MAPK-ERK regulator linked to a neurofibromatosis 1 (NF-1)-like human ..
  58. doi Expanding the phenotype of a neurofibromatosis type 1-like syndrome: a patient with a SPRED1 mutation and orbital manifestations
    Katherine A Lane
    Children s Hospital of Philadelphia, University of Pennsylvania Hospital System, Philadelphia, Pennsylvania 19104 4399, USA
    Ophthal Plast Reconstr Surg 25:399-401. 2009
    ..Further investigation revealed a loss of function mutation in SPRED1 on chromosome 15...
  59. pmc Down regulation of genes involved in T cell polarity and motility during the induction of heart allograft tolerance by allochimeric MHC I
    Wojciech Lisik
    Department of General and Transplantation Surgery, Warsaw Medical University, Warsaw, Poland
    PLoS ONE 4:e8020. 2009
    ..The immune response in allograft rejection involves a cascade of molecular events leading to the formation of immunological synapses between T cells and the antigen-presenting cells...
  60. doi Identification of LATS transcriptional targets in HeLa cells using whole human genome oligonucleotide microarray
    Stacy Visser
    Department of Pathology and Molecular Medicine, Queen s University, Kingston, ON, Canada K7L 3N6
    Gene 449:22-9. 2010
    ..known tumor suppressor genes and oncogenes such as CDKN1A, WISP2, SLIT2, TP53INP1, BIRC4BP, SPRY2, SPRY4, SPRED1, FAT4, and CYR61 were confirmed by qRT-PCR to be significantly differentially expressed...
  61. pmc TGF-beta modulates the functionality of tumor-infiltrating CD8+ T cells through effects on TCR signaling and Spred1 expression
    Maria Giovanna di Bari
    Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Drive, Room 8B09, MSC 1750, Bethesda, MD 20892, USA
    Cancer Immunol Immunother 58:1809-18. 2009
    ....
  62. doi Bcl-xL gene expression correlated with lower apoptotic cell numbers and shorter progression-free survival in PCFCL
    Razieh Soltani-Arabshahi
    INSERM, U728, Paris, F 75010, France
    J Invest Dermatol 129:1703-9. 2009
    ..an antiapoptotic member of the bcl-2 family, has been correlated with poor prognosis in nodal follicular lymphomas (NFLs)...
  63. doi A severe form of Noonan syndrome and autosomal dominant café-au-lait spots - evidence for different genetic origins
    Anna Maja Nyström
    Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden
    Acta Paediatr 98:693-8. 2009
    ..Therefore, investigations of the genetic cause of a severe phenotype in a patient with NS and the presence of multiple café-au-lait spots (CAL) spots in the patient and four members of the family were performed...
  64. ncbi Identification of endogenous phosphorylation sites of bovine medium and low molecular weight neurofilament proteins by tandem mass spectrometry
    Sarah Trimpin
    Department of Chemistry, Oregon State University, Corvallis, Oregon 97331, USA
    Biochemistry 43:2091-105. 2004
    ..while two sites (Ser(55) and Ser(472)) are consistent with the phosphorylations observed in other mammalian NFLs. The three sites, Ser(55), Ser(66), Ser(472), are heterogeneously phosphorylated...

Research Grants9

  1. Molecular Function of the Nf2 Tumor Suppressor, Merlin
    Andrea McClatchey; Fiscal Year: 2009
    ..The results of this study will yield important insight into the mechanism of Merlin function as a tumor suppressor and identify novel targets for therapeutic intervention of NF2. ..
  2. Molecular Function of the Nf2 Tumor Suppressor, Merlin
    Andrea I McClatchey; Fiscal Year: 2010
    ..These studies will advance our understanding of the molecular cause of NF2 and of the biology of ErbB receptors that have been widely implicated in human cancer. ..
  3. Comprehensive NF1 Mutational Analysis in Leukemia Cells
    Ludwine Messiaen; Fiscal Year: 2006
    ..abstract_text> ..