King's College London

Research portal

Professor Karen Steel

  1. Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation

    Corns, L. F., Johnson, S. L., Roberts, T., Ranatunga, K. M., Hendry, A., Ceriani, F., Safieddine, S., Steel, K. P., Forge, A., Petit, C., Furness, D. N., Kros, C. J. & Marcotti, W. 1 Dec 2018 In : Nature Communications. 9, 1, 4015

    Research output: Contribution to journalArticle

  2. Whole exome sequencing in adult-onset hearing loss reveals a high load of predicted pathogenic variants in known deafness-associated genes and identifies new candidate genes

    Lewis, M. A., Nolan, L. S., Cadge, B. A., Matthews, L. J., Schulte, B. A., Dubno, J. R., Steel, K. P. & Dawson, S. J. 4 Sep 2018 In : BMC medical genomics. 11, 1, 395

    Research output: Contribution to journalArticle

  3. miR-96 is required for normal development of the auditory hindbrain

    Schlüter, T., Berger, C., Rosengauer, E., Fieth, P., Krohs, C., Ushakov, K., Steel, K. P., Avraham, K. B., Hartmann, A. K., Felmy, F. & Nothwang, H. G. 1 Mar 2018 In : Human Molecular Genetics. 27, 5, p. 860-874 15 p.

    Research output: Contribution to journalArticle

  4. A large scale hearing loss screen reveals an extensive unexplored genetic landscape for auditory dysfunction

    Bowl, M. R. , Simon, M. M. , Ingham, N. J. , Greenaway, S. , Santos, L. , Cater, H. , Taylor, S. , Mason, J. , Kurbatova, N. , Pearson, S. , Bower, L. R. , Clary, D. A. , Meziane, H. , Reilly, P. , Minowa, O. , Kelsey, L. , Allen, S. , Clementson-Mobbs, S. , Codner, G. , Fray, M. & 181 others Gardiner, W., Joynson, R., Kenyon, J., Loeffler, J., Nell, B., Parker, A., Quwailid, D., Stewart, M., Walling, A., Zaman, R., Chen, C. K., Conte, N., Matthews, P., Relac, M., Tudose, I., Warren, J., Le Marchand, E., El Amri, A., El Fertak, L., Ennah, H., Ali-Hadji, D., Ayadi, A., Wattenhofer-Donze, M., Moulaert, D., Jacquot, S., André, P., Birling, M. C., Pavlovic, G., Lalanne, V., Lux, A., Riet, F., Mittelhaeuser, C., Bour, R., Guimond, A., Bam'Hamed, C., Leblanc, S., Vasseur, L., Selloum, M., Sorg, T., Ayabe, S., Furuse, T., Kaneda, H., Kobayashi, K., Masuya, H., Miura, I., Obata, Y., Suzuki, T., Tamura, M., Tanaka, N., Yamada, I., Yoshiki, A., Berberovic, Z., Bubshait, M., Cabezas, J., Carroll, T., Clark, G., Clarke, S., Creighton, A., Danisment, O., Eskandarian, M., Feugas, P., Gertsenstein, M., Guo, R., Hunter, J., Jacob, E., Lan, Q., Laurin, V., Law, N., MacMaster, S., Miller, D., Morikawa, L., Newbigging, S., Owen, C., Penton, P., Pereira, M., Qu, D., Shang, X., Sleep, G., Sohel, K., Tondat, S., Wang, Y., Vukobradovic, I., Zhu, Y., Chiani, F., Di Pietro, C., Di Segni, G., Ermakova, O., Ferrara, F., Fruscoloni, P., Gambadoro, A., Gastaldi, S., Golini, E., Sala, G. L., Mandillo, S., Marazziti, D., Massimi, M., Matteoni, R., Orsini, T., Pasquini, M., Raspa, M., Rauch, A., Rossi, G., Rossi, N., Putti, S., Scavizzi, F., Tocchini-Valentini, G. D., Beig, J., Bürger, A., Giesert, F., Graw, J., Kühn, R., Oritz, O., Schick, J., Seisenberger, C., Amarie, O., Garrett, L., Hölter, S. M., Zimprich, A., Aguilar-Pimentel, A., Beckers, J., Brommage, R., Calzada-Wack, J., Fuchs, H., Gailus-Durner, V., Lengger, C., Leuchtenberger, S., Maier, H., Marschall, S., Moreth, K., Neff, F., Östereicher, M. A., Rozman, J., Steinkamp, R., Stoeger, C., Treise, I., Stoeger, T., Yildrim, A. Ö., Eickelberg, O., Becker, L., Klopstock, T., Ollert, M., Busch, D. H., Schmidt-Weber, C., Bekeredjian, R., Zimmer, A., Rathkolb, B., Wolf, E., Klingenspor, M., Tocchini-Valentini, G. P., Gao, X., Bradley, A., Skarnes, W. C., Moore, M., Beaudet, A. L., Justice, M. J., Seavitt, J., Dickinson, M. E., Wurst, W., De Angelis, M. H., Herault, Y., Wakana, S., Nutter, L. M. J., Flenniken, A. M., McKerlie, C., Murray, S. A., Svenson, K. L., Braun, R. E., West, D. B., Lloyd, K. C. K., Adams, D. J., White, J., Karp, N., Flicek, P., Smedley, D., Meehan, T. F., Parkinson, H. E., Teboul, L. M., Wells, S., Steel, K. P., Mallon, A. M. & Brown, S. D. M. 1 Dec 2017 In : Nature Communications. 8, 1, 886

    Research output: Contribution to journalArticle

  5. On the role of ephrinA2 in auditory function

    Ingham, N. J., Steel, K. P. & Drescher, U. 5 Apr 2017 In : Hearing Research. 0, 0

    Research output: Contribution to journalArticle

  6. S1PR2 variants associated with auditory function in humans and endocochlear potential decline in mouse

    Ingham, N. J., Carlisle, F., Pearson, S., Lewis, M. A., Buniello, A., Chen, J., Isaacson, R. L., Pass, J., White, J. K., Dawson, S. J. & Steel, K. P. 7 Jul 2016 In : Scientific Reports. 6, 13 p., 28964

    Research output: Contribution to journalArticle

  7. Exploring regulatory networks of miR-96 in the developing inner ear

    Lewis, M. A., Buniello, A., Hilton, J. M., Zhu, F., Zhang, W. I., Evans, S., van Dongen, S., Enright, A. J. & Steel, K. P. 18 Mar 2016 In : Scientific Reports. 6, 23363

    Research output: Contribution to journalArticle

  8. Wbp2 is required for normal glutamatergic synapses in the cochlea and is crucial for hearing

    Buniello, A., Ingham, N. J., Lewis, M. A., Huma, A. C., Martinez-Vega, R., Varela-Nieto, I., Vizcay-Barrena, G., Fleck, R. A., Houston, O., Bardhan, T., Johnson, S. L., White, J. K., Yuan, H., Marcotti, W. & Steel, K. P. 8 Feb 2016 In : EMBO Molecular Medicine. 8, 3, p. 191-207 17 p.

    Research output: Contribution to journalArticle

  9. A Novel Locus Harbouring a Functional CD164 Nonsense Mutation Identified in a Large Danish Family with Nonsyndromic Hearing Impairment

    Nyegaard, M. , Rendtorff, N. D. , Nielsen, M. S. , Corydon, T. J. , Demontis, D. , Starnawska, A. , Hedemand, A. , Buniello, A. , Niola, F. , Overgaard, M. T. , Leal, S. M. , Ahmad, W. , Wikman, F. P. , Petersen, K. B. , Crüger, D. G. , Oostrik, J. , Kremer, H. , Tommerup, N. , Frödin, M. , Steel, K. P. & 2 others Tranebjærg, L. & Børglum, A. D. 1 Jul 2015 In : PL o S Genetics. 11, 7, e1005386

    Research output: Contribution to journalArticle

  10. OSBPL2 encodes a protein of inner and outer hair cell stereocilia and is mutated in autosomal dominant hearing loss (DFNA67)

    Thoenes, M., Zimmermann, U., Ebermann, I., Ptok, M., Lewis, M. A., Thiele, H., Morlot, S., Hess, M. M., Gal, A., Eisenberger, T., Bergmann, C., Nürnberg, G., Nürnberg, P., Steel, K. P., Knipper, M. & Bolz, H. J. 10 Feb 2015 In : Orphanet Journal Of Rare Diseases. 10, 1, 15

    Research output: Contribution to journalArticle

  11. ILDR1 null mice, a model of human deafness DFNB42, show structural aberrations of tricellular tight junctions and degeneration of auditory hair cells

    Morozko, E. L., Nishio, A., Ingham, N. J., Chandra, R., Fitzgerald, T., Martelletti, E., Borck, G., Wilson, E., Riordan, G. P., Wangemann, P., Forge, A., Steel, K. P., Liddle, R. A., Friedman, T. B. & Belyantseva, I. A. 1 Feb 2015 In : Human Molecular Genetics. 24, 3, p. 609-624 16 p., ddu474

    Research output: Contribution to journalArticle

  12. Salt-inducible kinase 3, SIK3, is a new gene associated with hearing

    Wolber, L. E., Girotto, G., Buniello, A., Vuckovic, D., Pirastu, N., Lorente-Cánovas, B., Rudan, I., Hayward, C., Polasek, O., Ciullo, M., Mangino, M., Steves, C., Concas, M. P. I., Cocca, M., Spector, T. D., Gasparini, P., Steel, K. P. & Williams, F. M. K. 1 Dec 2014 In : Human Molecular Genetics. 23, 23, p. 6407-6418 12 p.

    Research output: Contribution to journalArticle

  13. Identification of genes important for cutaneous function revealed by a large scale reverse genetic screen in the mouse

    DiTommaso, T., Jones, L. K., Cottle, D. L., Gerdin, A-K., Vancollie, V. E., Watt, F. M., Ramirez-Solis, R., Bradley, A., Steel, K. P., Sundberg, J. P., White, J. K., Smyth, I. M. & WTSI Mouse Genetics Program 23 Oct 2014 In : PLoS genetics. 10, 10, 12 p., e1004705

    Research output: Contribution to journalArticle

  14. Novel skin phenotypes revealed by a genome-wide mouse reverse genetic screen

    Liakath-Ali, K., Vancollie, V. E., Heath, E., Smedley, D. P., Estabel, J., Sunter, D., Ditommaso, T., White, J. K., Ramirez-Solis, R., Smyth, I., Steel, K. P. & Watt, F. M. 11 Apr 2014 In : Nature Communications. 5, N/A, p. N/A 13 p., 3540

    Research output: Contribution to journalArticle

  15. A reduction in Ptprq associated with specific features of the deafness phenotype of the miR-96 mutant mouse diminuendo

    Chen, J., Johnson, S. L., Lewis, M. A., Hilton, J. M., Huma, A., Marcotti, W. & Steel, K. P. Mar 2014 In : European Journal of Neuroscience. 39, 5, p. 744-756 13 p.

    Research output: Contribution to journalArticle

  16. Linkage study and exome sequencing identify a BDP1 mutation associated with hereditary hearing loss

    Girotto, G., Abdulhadi, K., Buniello, A., Vozzi, D., Licastro, D., D'Eustacchio, A., Vuckovic, D., Alkowari, M. K., Steel, K. P., Badii, R. & Gasparini, P. 2 Dec 2013 In : PL o S One . 8, 12, e80323

    Research output: Contribution to journalArticle

  17. Genome-wide Generation and Systematic Phenotyping of Knockout Mice Reveals New Roles for Many Genes

    White, J. K. , Gerdin, A-K. , Karp, N. A. , Ryder, E. , Buljan, M. , Bussell, J. N. , Salisbury, J. , Clare, S. , Ingham, N. J. , Podrini, C. , Houghton, R. , Estabel, J. , Bottomley, J. R. , Melvin, D. G. , Sunter, D. , Adams, N. C. , Sanger Institute Mouse Genetics Project, Tannahill, D. , Logan, D. W. , Macarthur, D. G. & 11 others Flint, J., Mahajan, V. B., Tsang, S. H., Smyth, I., Watt, F. M., Skarnes, W. C., Dougan, G., Adams, D. J., Ramirez-Solis, R., Bradley, A. & Steel, K. P. 18 Jul 2013 In : Cell. 154, 2, p. 452-464 13 p.

    Research output: Contribution to journalArticle

  18. Mouse large-scale phenotyping initiatives: overview of the European Mouse Disease Clinic (EUMODIC) and of the Wellcome Trust Sanger Institute Mouse Genetics Project

    Ayadi, A. , Birling, M-C. , Bottomley, J. , Bussell, J. , Fuchs, H. , Fray, M. , Gailus-Durner, V. , Greenaway, S. , Houghton, R. , Karp, N. , Leblanc, S. , Lengger, C. , Maier, H. , Mallon, A-M. , Marschall, S. , Melvin, D. , Morgan, H. , Pavlovic, G. , Ryder, E. , Skarnes, W. C. & 15 others Selloum, M., Ramirez-Solis, R., Sorg, T., Teboul, L., Vasseur, L., Walling, A., Weaver, T., Wells, S., White, J. K., Bradley, A., Adams, D. J., Steel, K., de Angelis, M. H., Brown, S. D. & Herault, Y. Oct 2012 In : Mammalian Genome. 23, 9-10, p. 600-610 11 p.

    Research output: Contribution to journalArticle

  19. A Cornucopia of Candidates for Deafness

    Lewis, M. A. & Steel, K. 31 Aug 2012 In : Cell. 150, 5, p. 879-881 4 p.

    Research output: Contribution to journalEditorial

  20. Atp6v0a4 knockout mouse is a model of distal renal tubular acidosis with hearing loss, with additional extrarenal phenotype

    Norgett, E. E., Lorente-Canovas, B., Golder, Z. J., Ingham, N., Steel, K. & Frankl, F. E. K. 21 Aug 2012 In : Proceedings of the National Academy of Sciences of the United States of America. 109, 34, p. 13775-13780 6 p.

    Research output: Contribution to journalArticle

  21. The Role of Sphingosine-1-Phosphate Transporter Spns2 in Immune System Function

    Nijnik, A., Clare, S., Hale, C., Chen, J., Raisen, C., Mottram, L., Lucas, M., Estabel, J., Ryder, E., Adissu, H., Adams, N. C., Ramirez-Solis, R., White, J. K., Steel, K., Dougan, G., Hancock, R. E. W. & Sanger Mouse Genetics Project 1 Jul 2012 In : Journal of Immunology. 189, 1, p. 102-111 10 p.

    Research output: Contribution to journalArticle

  22. Specific expression of Kcna10, Pxn and Odf2 in the organ of Corti

    Carlisle, F. A., Steel, K. & Lewis, M. A. May 2012 In : GENE EXPRESSION PATTERNS. 12, 5-6, p. 172-179 8 p.

    Research output: Contribution to journalArticle

  23. Auditory Function in the Tc1 Mouse Model of Down Syndrome Suggests a Limited Region of Human Chromosome 21 Involved in Otitis Media

    Kuhn, S., Ingham, N., Pearson, S., Gribble, S. M., Clayton, S., Steel, K. & Marcotti, W. 14 Feb 2012 In : PloS one. 7, 2, p. - 9 p., e31433

    Research output: Contribution to journalArticle

  24. Comprehensive sequence analysis of nine Usher syndrome genes in the UK National Collaborative Usher Study

    Stabej, P. L. Q., Saihan, Z., Rangesh, N., Steele-Stallard, H. B., Ambrose, J., Coffey, A., Emmerson, J., Haralambous, E., Hughes, Y., Steel, K., Luxon, L. M., Webster, A. R. & Bitner-Glindzicz, M. Jan 2012 In : Journal of Medical Genetics. 49, 1, p. 27-36 10 p.

    Research output: Contribution to journalArticle

  25. Mouse genetics for studying mechanisms of deafness and more: an interview with Karen Steel

    Steel, K. Nov 2011 In : Disease Models & Mechanisms. 4, 6, p. 716-718 3 p.

    Research output: Contribution to journalEditorial

  26. Exome sequencing identifies a missense mutation in Isl1 associated with low penetrance otitis media in dearisch mice

    Hilton, J. M., Lewis, M. A., Grati, M., Ingham, N., Pearson, S., Laskowski, R. A., Adams, D. J. & Steel, K. 21 Sep 2011 In : GENOME BIOLOGY. 12, 9, p. - 19 p., R90

    Research output: Contribution to journalArticle

  27. MUTATIONS IN THE USH1C GENE ASSOCIATED WITH SECTOR RETINITIS PIGMENTOSA AND HEARING LOSS

    Saihan, Z., Stabej, P. L. Q., Robson, A. G., Rangesh, N., Holder, G. E., Moore, A. T., Steel, K., Luxon, L. M., Bitner-Glindzicz, M. & Webster, A. R. Sep 2011 In : Retina. 31, 8, p. 1708-1716 9 p.

    Research output: Contribution to journalArticle

  28. The Usher 1B protein, MYO7A, is required for normal localization and function of the visual retinoid cycle enzyme, RPE65

    Lopes, V. S., Gibbs, D., Libby, R. T., Aleman, T. S., Welch, D. L., Lillo, C., Jacobson, S. G., Radu, R. A., Steel, K. & Williams, D. S. 1 Jul 2011 In : Human Molecular Genetics. 20, 13, p. 2560-2570 11 p.

    Research output: Contribution to journalArticle

  29. A missense mutation in Fgfr1 causes ear and skull defects in hush puppy mice

    Calvert, J. A., Dedos, S. G., Hawker, K., Fleming, M., Lewis, M. A. & Steel, K. Jun 2011 In : Mammalian Genome. 22, 5-6, p. 290-305 16 p.

    Research output: Contribution to journalArticle

  30. miR-96 regulates the progression of differentiation in mammalian cochlear inner and outer hair cells

    Kuhn, S., Johnson, S. L., Furness, D. N., Chen, J., Ingham, N., Hilton, J. M., Steffes, G., Lewis, M. A., Zampini, V., Hackney, C. M., Masetto, S., Holley, M. C., Steel, K. & Marcotti, W. 8 Feb 2011 In : Proceedings of the National Academy of Sciences of the United States of America. 108, 6, p. 2355-2360 6 p.

    Research output: Contribution to journalArticle

  31. The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice

    Schuetz, M., Scimemi, P., Majumder, P., De Siati, R. D., Crispino, G., Rodriguez, L., Bortolozzi, M., Santarelli, R., Seydel, A., Sonntag, S., Ingham, N., Steel, K., Willecke, K. & Mammano, F. 15 Dec 2010 In : Human Molecular Genetics. 19, 24, p. 4759-4773 15 p.

    Research output: Contribution to journalArticle

  32. MicroRNAs in mouse development and disease

    Lewis, M. A. & Steel, K. Sep 2010 In : SEMINARS IN CELL AND DEVELOPMENTAL BIOLOGY. 21, 7, p. 774-780 7 p.

    Research output: Contribution to journalLiterature review

  33. Emx2 and early hair cell development in the mouse inner ear

    Holley, M., Rhodes, C., Kneebone, A., Herde, M. K., Fleming, M. & Steel, K. 15 Apr 2010 In : Developmental Biology. 340, 2, p. 547-556 10 p.

    Research output: Contribution to journalArticle

  34. Study of smell and reproductive organs in a mouse model for CHARGE syndrome

    Bergman, J. E. H., Bosman, E. A., van Ravenswaaij-Arts, C. M. A. & Steel, K. Feb 2010 In : European Journal of Human Genetics. 18, 2, p. 171-177 7 p.

    Research output: Contribution to journalArticle

  35. Great vessel development requires biallelic expression of Chd7 and Tbx1 in pharyngeal ectoderm in mice

    Randall, V., McCue, K., Roberts, C., Kyriakopoulou, V., Beddow, S., Barrett, A. N., Vitelli, F., Prescott, K., Shaw-Smith, C., Devriendt, K., Bosman, E., Steffes, G., Steel, K. P., Simrick, S., Basson, M. A., Illingworth, E. & Scambler, P. J. 2 Nov 2009 In : The Journal of Clinical investigation. 119, 11, p. 3301 - 3310 10 p.

    Research output: Contribution to journalArticle

  36. MyosinVIIa Interacts with Twinfilin-2 at the Tips of Mechanosensory Stereocilia in the Inner Ear

    Rzadzinska, A. K., Nevalainen, E. M., Prosser, H. M., Lappalainen, P. & Steel, K. P. 23 Sep 2009 In : PloS one. 4, 9, p. - 10 p., e7097

    Research output: Contribution to journalArticle

  37. An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice

    Lewis, M. A., Quint, E., Glazier, A. M., Fuchs, H., De Angelis, M. H., Langford, C., van Dongen, S., Abreu-Goodger, C., Piipari, M., Redshaw, N., Dalmay, T., Angel Moreno-Pelayo, M., Enright, A. J. & Steel, K. May 2009 In : Nature Genetics. 41, 5, p. 614-618 5 p.

    Research output: Contribution to journalArticle

  38. Mutations in the seed region of human miR-96 are responsible for nonsyndromic progressive hearing loss

    Mencia, A., Modamio-Hoybjor, S., Redshaw, N., Morin, M., Mayo-Merino, F., Olavarrieta, L., Aguirre, L. A., del Castillo, I., Steel, K., Dalmay, T., Moreno, F. & Angel Moreno-Pelayo, M. May 2009 In : Nature Genetics. 41, 5, p. 609-613 5 p.

    Research output: Contribution to journalArticle

  39. Catweasel mice: A novel role for Six1 in sensory patch development and a model for branchio-oto-renal syndrome

    Bosman, E. A., Quint, E., Fuchs, H., de Angelis, M. H. & Steel, K. 15 Apr 2009 In : Developmental Biology. 328, 2, p. 285-296 12 p.

    Research output: Contribution to journalArticle

  40. Genetics, Gene Expression and Bioinformatics of the Pituitary Gland

    Davis, S. W., Potok, M. A., Brinkmeier, M. L., Carninci, P., Lyons, R. H., MacDonald, J. W., Fleming, M. T., Mortensen, A. H., Egashira, N., Ghosh, D., Steel, K., Osamura, R. Y., Hayashizaki, Y. & Camper, S. A. Apr 2009 In : Hormone Research. 71, S2, p. 101-115 15 p.

    Research output: Contribution to journalArticle

  41. Presence of interstereocilial links in waltzer mutants suggests Cdh23 is not essential for tip Link formation

    Rzadzinska, A. K. & Steel, K. 23 Jan 2009 In : Neuroscience. 158, 2, p. 365-368 4 p.

    Research output: Contribution to journalArticle

  42. A Myo6 Mutation Destroys Coordination between the Myosin Heads, Revealing New Functions of Myosin VI in the Stereocilia of Mammalian Inner Ear Hair Cells

    Hertzano, R., Shalit, E., Rzadzinska, A. K., Dror, A. A., Song, L., Ron, U., Tan, J. T., Shitrit, A. S., Fuchs, H., Hasson, T., Ben-Tal, N., Sweeney, H. L., de Angelis, M. H., Steel, K. & Avraham, K. B. Oct 2008 In : PL o S Genetics. 4, 10, p. - 14 p., e1000207

    Research output: Contribution to journalArticle

  43. The Novel Mouse Mutation Oblivion Inactivates the PMCA2 Pump and Causes Progressive Hearing Loss

    Spiden, S. L., Bortolozzi, M., Di Leva, F., de Angelis, M. H., Fuchs, H., Lim, D., Ortolano, S., Ingham, N. J., Brini, M., Carafoli, E., Mammano, F. & Steel, K. Oct 2008 In : PL o S Genetics. 4, 10, 12 p., e1000238

    Research output: Contribution to journalArticle

  44. Usher syndromes due to MYO7A, PCDH15, USH2A or GPR98 mutations share retinal disease mechanism

    Jacobson, S. G., Cideciyan, A. V., Aleman, T. S., Sumaroka, A., Roman, A. J., Gardner, L. M., Prosser, H. M., Mishra, M., Bech-Hansen, N. T., Herrera, W., Schwartz, S. B., Liu, X-Z., Kimberling, W. J., Steel, K. & Williams, D. S. 1 Aug 2008 In : Human Molecular Genetics. 17, 15, p. 2405-2415 11 p.

    Research output: Contribution to journalArticle

  45. Mosaic complementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereocilia

    Prosser, H. M., Rzadzinska, A. K., Steel, K. & Bradley, A. Mar 2008 In : Molecular and Cellular Biology. 28, 5, p. 1702-1712 11 p.

    Research output: Contribution to journalArticle

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