Research Topics
Genomes and Genes
| Matthew W KelleySummaryAffiliation: National Institutes of Health Country: USA Publications
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Detail Information
Publications
Leading Wnt down a PCP path: Cthrc1 acts as a coreceptor in the Wnt-PCP pathwayMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD 20892, USA
Dev Cell 15:7-8. 2008..A paper by Yamamoto et al. in this issue of Developmental Cell identifies collagen triple helix repeat containing 1 as a Wnt-binding cofactor that specifically activates the Wnt-PCP pathway...
Wnt activity guides facial branchiomotor neuron migration, and involves the PCP pathway and JNK and ROCK kinasesValerie Vivancos
MRC Centre for Developmental Neurobiology, King s College, Guy s Campus, London, SE1 1UL, UK
Neural Dev 4:7. 2009..In this study, we investigated a possible role of Wnts and the planar cell polarity (PCP) pathway in this process...
Regulation of cell fate in the sensory epithelia of the inner earMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 35 Convent Dr, Bethesda, Maryland 20892, USA
Nat Rev Neurosci 7:837-49. 2006..However, new methodologies have resulted in significant steps forward in our understanding of the molecular pathways that direct cells towards these cell fates...- Has hair cell loss MET its match?Matthew W Kelley
National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Porter Neuroscience Research Center, 35 Convent Drive, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 104:16400-1. 2007 - Cellular commitment and differentiation in the organ of CortiMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA
Int J Dev Biol 51:571-83. 2007..In this review I will present an overview of cochlear development and then discuss some of the most recent and enlightening results regarding the molecular mechanism underlying the formation of this remarkable structure... - Hair cell development: commitment through differentiationMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 35 Convent Drive, Bethesda, MA 20892, USA
Brain Res 1091:172-85. 2006..This review will summarize results from each of these developmental events and describe the molecular signaling pathways involved... - Regulation of cell fate and patterning in the developing mammalian cochleaMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA
Curr Opin Otolaryngol Head Neck Surg 17:381-7. 2009..In addition, targeted modulation of these same factors could be developed as therapies for hair cell regeneration... - Rbpj regulates development of prosensory cells in the mammalian inner earNorio Yamamoto
Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, NIDCD, NIH, Bethesda, MD 20892, USA
Dev Biol 353:367-79. 2011..These results suggest important roles for Rbpj and notch signaling in multiple aspects of inner ear development including prosensory cell maturation, cellular differentiation and survival... - Insulin-like growth factor signaling regulates the timing of sensory cell differentiation in the mouse cochleaTakayuki Okano
Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Porter Neuroscience Research Center, Bethesda, Maryland 20892, USA
J Neurosci 31:18104-18. 2011..The results also provide new insights regarding the pathological processes that underlie auditory defects in the absence of IGF signaling in both humans and mice... - Jxc1/Sobp, encoding a nuclear zinc finger protein, is critical for cochlear growth, cell fate, and patterning of the organ of cortiZheng Chen
Sections on Neurogenetics, Laboratory of Molecular Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, Maryland 20850, USA
J Neurosci 28:6633-41. 2008..Collectively, our data support a role for Jxc1 in controlling a critical step in cochlear growth, cell fate, and patterning of the organ of Corti... - Hedgehog signaling regulates sensory cell formation and auditory function in mice and humansElizabeth Carroll Driver
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurosci 28:7350-8. 2008..These results suggest that HH signaling plays a key role in the specification, size, and location of the prosensory domain, and therefore of hair cells, within the cochlea... - Sox2 signaling in prosensory domain specification and subsequent hair cell differentiation in the developing cochleaAlain Dabdoub
Section on Developmental Neuroscience, NIDCD, National Institutes of Health, Bethesda, MD 20892, USA
Proc Natl Acad Sci U S A 105:18396-401. 2008..These results demonstrate crucial and diverse roles for Sox2 in the development, specification, and maintenance of sensory cells within the cochlea... - Sox2 induces neuronal formation in the developing mammalian cochleaChandrakala Puligilla
Section on Developmental Neuroscience and Section on Synaptic Transmission, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurosci 30:714-22. 2010..Finally, we demonstrate that nonsensory cells within the cochlea retain neural competence through at least the early postnatal period... - Otic mesenchyme cells regulate spiral ganglion axon fasciculation through a Pou3f4/EphA4 signaling pathwayThomas M Coate
Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA
Neuron 73:49-63. 2012..These results indicate a model whereby Pou3f4 in the otic mesenchyme establishes an Eph/ephrin-mediated fasciculation signal that promotes inner radial bundle formation... - Fgf8 induces pillar cell fate and regulates cellular patterning in the mammalian cochleaBonnie E Jacques
Section on Developmental Neuroscience, Porter Neuroscience Research Center, 35 Convent Dr, Room 2A 100, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA
Development 134:3021-9. 2007..These results suggest that PCs might exist in a transient state of differentiation that makes them potential targets for regenerative therapies... - Inhibitors of differentiation and DNA binding (Ids) regulate Math1 and hair cell formation during the development of the organ of CortiJennifer M Jones
Section on Developmental Neuroscience, Porter Neuroscience Center, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurosci 26:550-8. 2006..These results indicate a key role for Ids in the regulation of expression of Math1 and hair cell differentiation in the developing cochlea... - Myosin II regulates extension, growth and patterning in the mammalian cochlear ductNorio Yamamoto
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, Bethesda, MD 20892, USA
Development 136:1977-86. 2009..Our results also provide insights into the cellular mechanisms that are required for the formation of highly ordered cellular patterns... - Fgf signaling regulates development and transdifferentiation of hair cells and supporting cells in the basilar papillaBonnie E Jacques
Laboratory of Cochlear Development, NIDCD, NIH, Porter Neuroscience Research Center, 35 Convent Dr, Room 2A 100, Bethesda, MD 20892 3729, USA
Hear Res 289:27-39. 2012..The ability of Fgf signaling to inhibit transdifferentiation suggests that its down-regulation may be essential for the initial steps of HC formation, as well as for the maintenance of SC phenotypes... - Building the world's best hearing aid; regulation of cell fate in the cochleaChandrakala Puligilla
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA
Curr Opin Genet Dev 19:368-73. 2009.... - Math1 regulates development of the sensory epithelium in the mammalian cochleaChad Woods
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Porter Neuroscience Research Center, Building 35, Bethesda, Maryland 20892, USA
Nat Neurosci 7:1310-8. 2004..These results show that Math1 functions in the developing cochlea to initiate both inductive and inhibitory signals that regulate the overall formation of the sensory epithelia... - Asymmetric localization of Vangl2 and Fz3 indicate novel mechanisms for planar cell polarity in mammalsMireille Montcouquiol
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurosci 26:5265-75. 2006..Together, our data support the idea that protein asymmetry plays an important role in the development of PCP, but the colocalization and interaction of Fz3 and Vangl2 suggests that novel PCP mechanisms exist in vertebrates... - Planar and vertical signals control cellular differentiation and patterning in the mammalian cochleaMireille Montcouquiol
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders National Institutes of Health, Rockville, Maryland 20850, USA
J Neurosci 23:9469-78. 2003..Finally, we demonstrate that the temporal gradient of cellular differentiation is not dependent on planar signals within the developing sensory epithelium... - Transfection of mouse cochlear explants by electroporationElizabeth C Driver
National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA
Curr Protoc Neurosci . 2010..While the protocol is largely focused on embryonic cochlea, the same basic protocol can be used on cochleae from mice as old as P5... - Disruption of fibroblast growth factor receptor 3 signaling results in defects in cellular differentiation, neuronal patterning, and hearing impairmentChandrakala Puligilla
Section on Developmental Neuroscience, NIDCD NIH, Bethesda, Maryland 20892, USA
Dev Dyn 236:1905-17. 2007.... - Specification of cell fate in the mammalian cochleaElizabeth C Driver
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
Birth Defects Res C Embryo Today 87:212-21. 2009..A number of different signaling pathways and transcription factors are known to be necessary for these developmental processes; in this review, we will summarize these results with an emphasis on recent findings... - Cellular growth and rearrangement during the development of the mammalian organ of CortiErynn McKenzie
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, Maryland, USA
Dev Dyn 229:802-12. 2004..These results are consistent with the hypothesis that convergence and extension plays a role in cellular patterning within the organ of Corti... - Determination and commitment of mechanosensory hair cellsMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Room 2B 44, Rockville, MD 20850, USA
ScientificWorldJournal 2:1079-94. 2002.... - Identification of Vangl2 and Scrb1 as planar polarity genes in mammalsMireille Montcouquiol
Section on Developmental Neuroscience, NIDCD, National Institutes of Health, Rockville, Maryland 20850, USA
Nature 423:173-7. 2003..These results demonstrate a role for the PCP pathway in planar polarization in mammals, and identify Scrb1 as a PCP gene... - Fibroblast growth factor receptor 3 regulates microtubule formation and cell surface mechanical properties in the developing organ of CortiKatherine B Szarama
Auditory Mechanics Section, Laboratory of Cellular Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD, USA
Bioarchitecture 2:214-9. 2012..We conclude that microtubule formation is a major downstream effector of Fgf-receptor 3, and suggest this pathway impacts the formation of fluid spaces in the organ of Corti... - Thyroid hormone increases fibroblast growth factor receptor expression and disrupts cell mechanics in the developing organ of cortiKatherine B Szarama
Section on Developmental Neuroscience, Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD, USA
BMC Dev Biol 13:6. 2013..The mammalian cochlea is an intriguing system to examine these mechanisms, as cellular structure plays a key role in tissue development, and thyroid hormone is required for the maturation of the cochlea in the first postnatal week... - Molecular mechanisms of inner ear developmentDoris K Wu
National Institute on Deafness and Other Communication Disorders, Rockville, Maryland 20850, USA
Cold Spring Harb Perspect Biol 4:a008409. 2012..This chapter focuses on molecular mechanisms of inner ear development derived from studies of model organisms... - Examining planar cell polarity in the mammalian cochleaHelen May-Simera
Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD, USA
Methods Mol Biol 839:157-71. 2012.... - Cell adhesion molecules during inner ear and hair cell development, including notch and its ligandsMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, Maryland 20850, USA
Curr Top Dev Biol 57:321-56. 2003..At the same time, the generation of new transgenic and molecular technologies promises to provide researchers with new tools to examine the specific effects of different adhesion molecules during inner ear development... - Planar cell polarity and a potential role for a Wnt morphogen gradient in stereociliary bundle orientation in the mammalian inner earAlain Dabdoub
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurobiol 64:446-57. 2005..Furthermore, we review evidence for the role of Wnt signaling, and the possible generation of a Wnt gradient, in planar polarity... - Development of tonotopy in the auditory peripheryZoë F Mann
Laboratory of Cochlear Development, NIDCD, NIH, Bethesda, MD 20892, USA
Hear Res 276:2-15. 2011..However, recent studies of developing systems, including the inner ear provide some clues regarding the signalling pathways that may be instructive for the formation of a tonotopic axis... - Stem cell therapy for the inner ear: recent advances and future directionsTakayuki Okano
National Institutes of Health, Bethesda, MD 20892, USA
Trends Amplif 16:4-18. 2012..Subsequent sections discuss the possible use of endogenous inner ear stem cells to induce repair as well as the initial studies aimed at transplanting stem cells into the ear... - Combining Cep290 and Mkks ciliopathy alleles in mice rescues sensory defects and restores ciliogenesisRivka A Rachel
Neurobiology Neurodegeneration and Repair Laboratory, National Eye Institute, NIH, Bethesda, Maryland 20892, USA
J Clin Invest 122:1233-45. 2012..Amelioration of the sensory phenotypes caused by specific mutations in one protein by removal of an interacting domain/protein suggests a possible novel approach for treating human ciliopathies... - Noncanonical Wnt signaling and neural polarityMireille Montcouquiol
Section on Developmental Neuroscience, NIDCD, NIH, Bethesda, Maryland 20982, USA
Annu Rev Neurosci 29:363-86. 2006.... - Fibroblast growth factor signaling regulates pillar cell development in the organ of cortiKristen L Mueller
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, Maryland 20850, USA
J Neurosci 22:9368-77. 2002..Moreover, the position of the pillar cells appears to be determined by the activation of FGFR3 in a subset of the progenitor cells that initially express this receptor... - The Atoh1-lineage gives rise to hair cells and supporting cells within the mammalian cochleaElizabeth Carroll Driver
Laboratory of Cochlear Development, National Institute on Deafness and Other Communications Disorders, NIH, Bethesda, MD 20892, USA
Dev Biol 376:86-98. 2013..Our results demonstrate that the Atoh1-lineage is established early in cochlear development, but also show that expression of Atoh1 does not absolutely result in commitment to a HC fate... - Cytoskeletal changes in actin and microtubules underlie the developing surface mechanical properties of sensory and supporting cells in the mouse cochleaKatherine B Szarama
Section on Auditory Mechanics, Laboratory of Cellular Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA
Development 139:2187-97. 2012..Finally, this study found that the fibroblast growth factor signaling pathway is necessary for the developmental time course of cell surface mechanical properties, in part owing to the effects on microtubule structure... - Wnt signaling mediates reorientation of outer hair cell stereociliary bundles in the mammalian cochleaAlain Dabdoub
Section on Developmental Neuroscience, NIDCD, National Institutes of Health, Rockville, MD 20850, USA
Development 130:2375-84. 2003..We propose that Wnt signaling across the region of developing outer hair cells gives rise to planar polarity in the mammalian cochlea... - Analysis of nuclear receptor function in the mouse auditory systemMatthew W Kelley
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, Maryland 20850, USA
Methods Enzymol 364:426-48. 2003 - Cilia, Wnt signaling, and the cytoskeletonHelen L May-Simera
Laboratory of Cochlear Development, National Institute on Deafness and Other Communication Disorders, NIH, 35 Convent Drive, Bethesda, MD 20892, USA
Cilia 1:7. 2012..This review summarizes recent advances in our understanding of the involvement of cilia and basal body proteins in Wnt signaling pathways... - Planar cell polarity in the inner earHelen May-Simera
Laboratory of Cochlear Development, NIDCD, NIH, Bethesda, Maryland, USA
Curr Top Dev Biol 101:111-40. 2012..Finally, the presence of shortened cochleae in many PCP mouse mutants has revealed an additional role for the PCP pathway in the development of the auditory system... - Knock-in mouse model for resistance to thyroid hormone (RTH): an RTH mutation in the thyroid hormone receptor beta gene disrupts cochlear morphogenesisAndrew J Griffith
Hearing Section, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, MD 20850, USA
J Assoc Res Otolaryngol 3:279-88. 2002..Comparison with the previously described phenotype of a Thrb -/- knockout strain suggests that Thrb(PV) disrupts the function of other genes that are critical for development and/or maintenance of these structures... - Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pairQiang Xu
Department of Molecular Biology and Genetics, Howard Hughes Medical Institute, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Cell 116:883-95. 2004..These data define a Norrin-Fz4 signaling system that plays a central role in vascular development in the eye and ear, and they indicate that ligands unrelated to Wnts can act through Fz receptors... - Transcription profiling of inner ears from Pou4f3(ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness geneRonna Hertzano
Department of Human Genetics and Molecular Medicine, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
Hum Mol Genet 13:2143-53. 2004..These results identify Gfi1 as the first downstream target of a hair cell specific transcription factor and suggest that outer hair cell degeneration in Pou4f3 mutants is largely or entirely a result of the loss of expression of Gfi1... - Hearing loss and retarded cochlear development in mice lacking type 2 iodothyronine deiodinaseLily Ng
Department of Human Genetics and Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
Proc Natl Acad Sci U S A 101:3474-9. 2004..Thus, D2 is essential for hearing, and the results suggest that this hormone-activating enzyme confers on the cochlea the ability to stimulate its own T3 response at a critical developmental period... - Lhx3, a LIM domain transcription factor, is regulated by Pou4f3 in the auditory but not in the vestibular systemRonna Hertzano
Department of Human Molecular Genetics and Biochemistry, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
Eur J Neurosci 25:999-1005. 2007..This is the first example of a hair cell-specific gene expressed both in auditory and in vestibular hair cells, with differential regulation of expression in these two closely related systems... - From placode to polarization: new tunes in inner ear developmentKate F Barald
Department of Cell and Developmental Biology, Program in Neuroscience, Cell and Molecular Biology Program, University of Michigan Medical School, Ann Arbor, MI 48109-0616, USA
Development 131:4119-30. 2004..This review summarizes studies addressing several key issues that shape our current thinking about inner ear development, with particular emphasis on early patterning events, sensory hair cell specification and planar cell polarity... - Myosin VI and VIIa distribution among inner ear epithelia in diverse fishesAllison B Coffin
Department of Biology, University of Maryland, College Park, MD 20742, USA
Hear Res 224:15-26. 2007..The lack of myo6 in specific American shad utricular hair bundles indicates a unique quality of these cells among fishes, perhaps relating to ultrasound detection capability that is found in this species...