STRC

Summary

Gene Symbol: STRC
Description: stereocilin
Alias: DFNB16
Species: human

Top Publications

  1. ncbi Mutations in a new gene encoding a protein of the hair bundle cause non-syndromic deafness at the DFNB16 locus
    E Verpy
    Unité de Génétique des Déficits Sensoriels, CNRS URA 1968, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
    Nat Genet 29:345-9. 2001
  2. ncbi RT-PCR analysis of Tecta, Coch, Eya4 and Strc in mouse cochlear explants
    Yukihide Maeda
    Molecular Otolaryngology Research Laboratory, Department of Otolaryngology, The University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA
    Neuroreport 16:361-5. 2005
  3. pmc Genome-wide SNP genotyping identifies the Stereocilin (STRC) gene as a major contributor to pediatric bilateral sensorineural hearing impairment
    Lauren J Francey
    The Division of Human Genetics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Am J Med Genet A 158:298-308. 2012
  4. pmc Diagnostic application of targeted resequencing for familial nonsyndromic hearing loss
    Byung Yoon Choi
    Department of Otorhinolaryngology, Seoul National University Bundang Hospital, Seongnam, Korea
    PLoS ONE 8:e68692. 2013
  5. doi Enhanced normal short-term human myelopoiesis in mice engineered to express human-specific myeloid growth factors
    Paul H Miller
    Terry Fox Laboratory, BC Cancer Agency, Vancouver, BC, Canada
    Blood 121:e1-4. 2013
  6. pmc Novel molecular pathways elicited by mutant FGFR2 may account for brain abnormalities in Apert syndrome
    Erika Yeh
    Human Genome Center, Department of Genetics and Evolutive Biology, Institute of Bioscience, University of Sao Paulo, Sao Paulo, SP, Brazil
    PLoS ONE 8:e60439. 2013
  7. pmc Genetic testing for hearing loss in the United States should include deletion/duplication analysis for the deafness/infertility locus at 15q15.3
    Nicole Hoppman
    Department of Laboratory Medicine and Pathology, Mayo Clinic, 971 Hilton, 200 1st St SW, Rochester, MN, 55905, USA
    Mol Cytogenet 6:19. 2013
  8. pmc A phylomedicine approach to understanding the evolution of auditory sensory perception and disease in mammals
    John D Kirwan
    UCD School of Biology and Environmental Science and UCD Conway Institute of Biomolecular and Biomedical Research, University College Dublin Dublin, Ireland
    Evol Appl 6:412-22. 2013
  9. doi Existence and stability criteria for phase-locked modes in ring neural networks based on the spike time resetting curve method
    Sorinel Adrian Oprisan
    College of Charleston, Physics and Astronomy Department, 58 Coming Street, Room 101, Charleston, SC 29424, USA
    J Theor Biol 262:232-44. 2010
  10. pmc Synchrony with shunting inhibition in a feedforward inhibitory network
    Sachin S Talathi
    J Crayton Pruitt Family Department of Biomedical Engineering, University of Florida, Gainesville, FL 32611, USA
    J Comput Neurosci 28:305-21. 2010

Research Grants

  1. CLONING TWO NEW DEAFNESS MUTATIONS
    Kenneth Johnson; Fiscal Year: 2004

Scientific Experts

  • Sachin S Talathi
  • Nicole Hoppman
  • Leonard D Shultz
  • Thorsten Wolf
  • Luca Jovine
  • Sorinel Adrian Oprisan
  • Elisabeth Verpy
  • Jana Michalová
  • Kenneth Johnson
  • Harry Taber
  • Kent Christopherson
  • Erika Yeh
  • Paul H Miller
  • John D Kirwan
  • Byung Yoon Choi
  • Lauren J Francey
  • Connie J Eaves
  • Marlene Tacke
  • Oksana Zavidij
  • Manfred Schmidt
  • Claudia R Ball
  • Christof von Kalle
  • Hanno Glimm
  • Sylvia Fessler
  • J Knijnenburg
  • Oliver Christ
  • Yuzhou Zhang
  • Richard J H Smith
  • Dmitri D Pervouchine
  • Yukihide Maeda
  • Maria Bitner-Glindzicz
  • Nivaldo Alonso
  • Bong Jik Kim
  • R Keith Humphries
  • Kiran Dhillon
  • Gabrielle Rabu
  • Daniele Y Sunaga
  • Ah Reum Kim
  • Seung Ha Oh
  • Kalina T J Davies
  • Xueyan Zhou
  • Michael Bekaert
  • Jungsoo Gim
  • Gregory Holmes
  • Kyu Hee Han
  • Joo Hyun Park
  • Roberto D Fanganiello
  • Emma C Teeling
  • Hamilton Matushita
  • David J H F Knapp
  • Hyo Sang Kim
  • Woong Yang Park
  • Philip A Beer
  • Ethylin W Jabs
  • Maria Rita Passos-Bueno
  • Gibeom Park
  • Taesung Park
  • Jennifer M Commins
  • Alice M S Cheung
  • Shabnam Rostamirad
  • Stephen J Rossiter
  • Yingli Wang
  • Kátia M Rocha
  • M Mangino
  • Donna Berrodin
  • Nancy B Spinner
  • Yi Sun
  • H Glimm
  • Laura K Conlin
  • Michal Sagi
  • Chaim Landau
  • Ian D Krantz
  • Joe Glessner
  • Margaret Kenna
  • Dinah Clark
  • Hakon Hakonarson
  • Hanna E Kadesch
  • Heidi L Rehm
  • Chaim Jalas
  • Silke Klingenberg
  • Birgitta Maurer
  • Friederike Herbst
  • T Lucas
  • S A J Lesnik Oberstein
  • C A L Ruivenkamp
  • K Frei
  • H J Tanke
  • K Szuhai
  • A C J Gijsbers
  • M Villamar

Detail Information

Publications29

  1. ncbi Mutations in a new gene encoding a protein of the hair bundle cause non-syndromic deafness at the DFNB16 locus
    E Verpy
    Unité de Génétique des Déficits Sensoriels, CNRS URA 1968, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
    Nat Genet 29:345-9. 2001
    ..29 coding exons in two families affected by autosomal recessive non-syndromal sensorineural deafness linked to the DFNB16 locus...
  2. ncbi RT-PCR analysis of Tecta, Coch, Eya4 and Strc in mouse cochlear explants
    Yukihide Maeda
    Molecular Otolaryngology Research Laboratory, Department of Otolaryngology, The University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA
    Neuroreport 16:361-5. 2005
    Tecta, Coch, Eya4 and Strc are mouse orthologs of four human deafness-associated genes. Their expression is markedly restricted to specific cell types in cochleae. Cochleae were dissected on embryonic day 15 and cultured in vitro...
  3. pmc Genome-wide SNP genotyping identifies the Stereocilin (STRC) gene as a major contributor to pediatric bilateral sensorineural hearing impairment
    Lauren J Francey
    The Division of Human Genetics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Am J Med Genet A 158:298-308. 2012
    ..After identifying 8 mild-moderate NBSNHI probands with a Chr15q15.3 deletion encompassing the Stereocilin (STRC) gene amongst this cohort, sequencing of STRC was undertaken in these probands as well as 50 probands and 14 ..
  4. pmc Diagnostic application of targeted resequencing for familial nonsyndromic hearing loss
    Byung Yoon Choi
    Department of Otorhinolaryngology, Seoul National University Bundang Hospital, Seongnam, Korea
    PLoS ONE 8:e68692. 2013
    ..by the targeted resequencing were distributed in nine genes such as WFS1, COCH, EYA4, MYO6, GJB3, COL11A2, OTOF, STRC and MYO3A, most of which were private...
  5. doi Enhanced normal short-term human myelopoiesis in mice engineered to express human-specific myeloid growth factors
    Paul H Miller
    Terry Fox Laboratory, BC Cancer Agency, Vancouver, BC, Canada
    Blood 121:e1-4. 2013
    ..were heterogeneously distributed, validating the blood as a more representative measure of transplanted STRC activity...
  6. pmc Novel molecular pathways elicited by mutant FGFR2 may account for brain abnormalities in Apert syndrome
    Erika Yeh
    Human Genome Center, Department of Genetics and Evolutive Biology, Institute of Bioscience, University of Sao Paulo, Sao Paulo, SP, Brazil
    PLoS ONE 8:e60439. 2013
    ..Strikingly, we validated Strc (stereocilin) in newborn Fgfr2(S252W/+) mouse brain...
  7. pmc Genetic testing for hearing loss in the United States should include deletion/duplication analysis for the deafness/infertility locus at 15q15.3
    Nicole Hoppman
    Department of Laboratory Medicine and Pathology, Mayo Clinic, 971 Hilton, 200 1st St SW, Rochester, MN, 55905, USA
    Mol Cytogenet 6:19. 2013
    ..Syndrome; DIS) is a contiguous gene deletion syndrome resulting from homozygous deletion of the CATSPER2 and STRC genes on chromosome 15q15.3. Females with DIS have only hearing loss and are fertile...
  8. pmc A phylomedicine approach to understanding the evolution of auditory sensory perception and disease in mammals
    John D Kirwan
    UCD School of Biology and Environmental Science and UCD Conway Institute of Biomolecular and Biomedical Research, University College Dublin Dublin, Ireland
    Evol Appl 6:412-22. 2013
    ..Using cross-species comparisons of 11 'deafness' genes (Myo15, Ush1 g, Strc, Tecta, Tectb, Otog, Col11a2, Gjb2, Cldn14, Kcnq4, Pou3f4) across 69 evolutionary and ecologically divergent ..
  9. doi Existence and stability criteria for phase-locked modes in ring neural networks based on the spike time resetting curve method
    Sorinel Adrian Oprisan
    College of Charleston, Physics and Astronomy Department, 58 Coming Street, Room 101, Charleston, SC 29424, USA
    J Theor Biol 262:232-44. 2010
    ..phase-locked modes in ring neural networks of spiking neurons based on the open loop spike time resetting curve (STRC) and its almost equivalent counterpart-the phase resetting curve (PRC)...
  10. pmc Synchrony with shunting inhibition in a feedforward inhibitory network
    Sachin S Talathi
    J Crayton Pruitt Family Department of Biomedical Engineering, University of Florida, Gainesville, FL 32611, USA
    J Comput Neurosci 28:305-21. 2010
    ..in the presence of heterogeneity, we develop a general theoretical framework using spike time response curves (STRC's) to study patterns of synchrony in a simple network of two unidirectionally coupled interneurons (UCI network) ..
  11. doi Hematopoietic activity of human short-term repopulating cells in mobilized peripheral blood cell transplants is restricted to the first 5 months after transplantation
    Oksana Zavidij
    Department of Translational Oncology, National Center for Tumor Diseases and German Cancer Research Center, Heidelberg, Germany
    Blood 115:5023-5. 2010
    ..durability and lineage contribution of STRCs, we compared repopulation kinetics of mobilized peripheral blood (high STRC content) with cord blood transplants (low STRC content) in long-lived NOD...
  12. pmc Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane
    Elisabeth Verpy
    Unite de Genetique et Physiologie de l Audition, Institut Pasteur, Paris, France
    J Comp Neurol 519:194-210. 2011
    Stereocilin is defective in a recessive form of deafness, DFNB16. We studied the distribution of stereocilin in the developing and mature mouse inner ear and analyzed the consequences of its absence in stereocilin-null (Strc(-/-)) mice ..
  13. doi Hematopoietic stem cells survive circulation arrest and reconstitute hematopoiesis in myeloablated mice
    Jana Michalová
    Center of Experimental Hematology and Institute of Pathological Physiology, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic
    Biol Blood Marrow Transplant 17:1273-81. 2011
    ..a higher sensitivity of proliferating hematopoietic progenitors to ischemia compared to repopulating cells (STRC and LTRC)...
  14. doi The inherent differentiation program of short-term hematopoietic repopulating cells changes during human ontogeny
    Marlene Tacke
    Institute of Molecular Medicine and Cell Research, Albert Ludwigs University, Freiburg, Germany
    Stem Cells Dev 19:621-8. 2010
    ..Compared to adult sources of hematopoietic cells, CB myeloidrestricted STRC-M showed a markedly reduced megakaryocytic and erythroid cell output in the quantitative xenotransplantation of ..
  15. doi A homozygous deletion of a normal variation locus in a patient with hearing loss from non-consanguineous parents
    J Knijnenburg
    Department of Molecular Cell Biology, zone S 1 P, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands
    J Med Genet 46:412-7. 2009
    ..The impact on disease of CNVs in the homozygous state may thus remain undetected and underestimated...
  16. doi Spike timing dependent plasticity promotes synchrony of inhibitory networks in the presence of heterogeneity
    Sachin S Talathi
    J Crayton Pruitt Family Department of Biomedical Engineering, University of Florida, Gainesville, FL 32611, USA
    J Comput Neurosci 25:262-81. 2008
    ..Using the method of spike time response curve (STRC), we show how iSTDP influences the dynamics of the coupled neurons, such that the pair synchronizes under ..
  17. pmc A new locus for non-syndromal, autosomal recessive, sensorineural hearing loss (DFNB16) maps to human chromosome 15q21-q22
    D A Campbell
    Molecular Medicine Unit, St James s University Hospital, Leeds, UK
    J Med Genet 34:1015-7. 1997
    ..We report the mapping of a novel locus for autosomal recessive, non-syndromal deafness (DFNB16) in three consanguineous families originating from Pakistan and the Middle East...
  18. pmc Deafness locus DFNB16 is located on chromosome 15q13-q21 within a 5-cM interval flanked by markers D15S994 and D15S132
    M Villamar
    Am J Hum Genet 64:1238-41. 1999
  19. pmc Previously undetected human hematopoietic cell populations with short-term repopulating activity selectively engraft NOD/SCID-beta2 microglobulin-null mice
    H Glimm
    Terry Fox Laboratory, British Columbia Cancer Agency, and Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada
    J Clin Invest 107:199-206. 2001
    ..myeloid-restricted STRCs were preferentially amplified (greater than tenfold) and, interestingly, both types of STRC were found to be selectively elevated in mobilized peripheral blood harvests...
  20. ncbi Mapping of a new autosomal dominant nonsyndromic hearing loss locus (DFNA30) to chromosome 15q25-26
    M Mangino
    CSS Mendel Institute, IRCCS, Rome, Italy
    Eur J Hum Genet 9:667-71. 2001
    ..This region does not overlap with DFNB16 locus but partially coincides with the otosclerosis (OTS) locus...
  21. ncbi Hereditary deafness and phenotyping in humans
    Maria Bitner-Glindzicz
    Unit of Clinical and Molecular Genetics, Institute of Child Health, London, UK
    Br Med Bull 63:73-94. 2002
    ..Exceptions to this include DFNB2 (MYO7A), DFNB8/10 (TMPRSS3) and DFNB16 (STRC) where age of onset may sometimes be later on in childhood, DFNB4 (SLC26A4) where there may be dilated ..
  22. pmc Sequence similarity between stereocilin and otoancorin points to a unified mechanism for mechanotransduction in the mammalian inner ear
    Luca Jovine
    Brookdale Department of Molecular, Cell and Developmental Biology, Mount Sinai School of Medicine, New York, NY 10029 6574, USA
    BMC Cell Biol 3:28. 2002
    ..Recently, otoancorin was suggested to be a mediator of gel attachment to nonsensory cells, but the molecular components of the interface between gels and sensory cells remain to be identified...
  23. ncbi Regulation of human short-term repopulating cell (STRC) engraftment in NOD/SCID mice by host CD122+ cells
    Leonard D Shultz
    The Jackson Laboratory, 600 Main Street, Bar Harbor, Maine 04609, USA
    Exp Hematol 31:551-8. 2003
    ..A previously unrecognized HSC in cord blood, termed short-term repopulating cell (STRC), has been identified using NOD/SCID B2m(null) mice...
  24. ncbi Low-dimensional maps encoding dynamics in entorhinal cortex and hippocampus
    Dmitri D Pervouchine
    Department of Mathematics and Statistics and Center for BioDynamics, Boston University, Boston, MA 02215, USA
    Neural Comput 18:2617-50. 2006
    ..We use spike time response curve methods (STRC), expanding that technique to three-cell networks and giving two different ways in which the analysis of the three-..
  25. pmc Sensorineural deafness and male infertility: a contiguous gene deletion syndrome
    Yuzhou Zhang
    Molecular Otolaryngology Research Laboratories, Department of Otolaryngology, University of Iowa, Iowa City, Iowa 52240, USA
    J Med Genet 44:233-40. 2007
    ..Syndromic hearing loss that results from contiguous gene deletions is uncommon. Deafness-infertility syndrome (DIS) is caused by large contiguous gene deletions at 15q15.3...
  26. ncbi Improved purification of hematopoietic stem cells based on their elevated aldehyde dehydrogenase activity
    Oliver Christ
    Terry Fox Laboratory, British Columbia Cancer Agency, Vancouver, BC, Canada
    Haematologica 92:1165-72. 2007
    ....
  27. doi The hen's egg test for micronucleus induction (HET-MN): novel analyses with a series of well-characterized substances support the further evaluation of the test system
    Thorsten Wolf
    Universitat Osnabruck, Fb8, Pharmakologie und Toxikologie, Albrechtstr 28, Osnabruck, Germany
    Mutat Res 650:150-64. 2008
    ..Negative results were obtained for azorubin (E122), orange G (OG) and starch (STRC)...
  28. pmc Structure and expression of the cytochrome aa3 regulatory gene ctaA of Bacillus subtilis
    J P Mueller
    Department of Microbiology and Immunology, Albany Medical College, New York 12208
    J Bacteriol 171:4979-86. 1989
    ..Postexponential expression was found to be dependent on the product of the strC gene. The expression of ctaA appears to be regulated in a growth stage-specific manner...

Research Grants9

  1. CLONING TWO NEW DEAFNESS MUTATIONS
    Kenneth Johnson; Fiscal Year: 2004
    ..genetic map relationships, the new mouse mutations may be homologous to the human nonsyndromic deafness genes DFNB16 and DFNA13...