Research Topics
Genomes and Genes
| Pierre A CoulombeSummaryAffiliation: Johns Hopkins Bloomberg School of Public Health Country: USA Publications
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Detail Information
Publications
Defining keratin protein function in skin epithelia: epidermolysis bullosa simplex and its aftermathPierre A Coulombe
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland 21205, USA
J Invest Dermatol 132:763-75. 2012....- Epidermolysis bullosa simplex: a paradigm for disorders of tissue fragilityPierre A Coulombe
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA
J Clin Invest 119:1784-93. 2009..Here we review how laboratory investigations centered on keratin biology have deepened our understanding of the etiology and pathophysiology of EB simplex and revealed novel avenues for its therapy... - Exploiting the keratin 17 gene promoter to visualize live cells in epithelial appendages of miceNicholas Bianchi
Predoctoral Program in Human Genetics, McKusick Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 212105, USA
Mol Cell Biol 25:7249-59. 2005..The compact mK17 gene promoter provides a novel tool for appendage-preferred gene expression and manipulation in transgenic mice... - Defining the properties of the nonhelical tail domain in type II keratin 5: insight from a bullous disease-causing mutationLi Hong Gu
Departments of Biological Chemistry and Dermatology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Mol Biol Cell 16:1427-38. 2005..These data provide a mechanistic basis for the cell fragility seen in individuals bearing the K5-1649delG allele, and point to the role of the C-terminal 41 residues in determining K5's assembly properties... - Overcoming functional redundancy to elicit pachyonychia congenita-like nail lesions in transgenic micePauline Wong
Department of Biological Chemistry, Johns Hopkins University School of Medicine, 725 North Wolfe St, Baltimore, MD 21205, USA
Mol Cell Biol 25:197-205. 2005..Our findings point to significant redundancy among the multiple keratins expressed in hair and nail, which can be related to the common ancestry, clustered organization, and sequence relatedness of specific keratin genes... - Keratin 17 promotes epithelial proliferation and tumor growth by polarizing the immune response in skinDaryle DePianto
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA
Nat Genet 42:910-4. 2010..Our findings establish an immunomodulatory role for Krt17 in Hh driven basaloid skin tumors that could impact additional tumor settings, psoriasis and wound repair... - Hairless triggers reactivation of hair growth by promoting Wnt signalingGerard M J Beaudoin
Kennedy Krieger Research Institute and Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Proc Natl Acad Sci U S A 102:14653-8. 2005..This work links HR and Wnt function, providing a model in which HR regulates the precise timing of Wnt signaling required for hair follicle regeneration... 
The co-repressor hairless has a role in epithelial cell differentiation in the skinJoanna M Zarach
Kennedy Krieger Research Institute, Baltimore, MD 21205, USA
Development 131:4189-200. 2004....- Keratin expression provides novel insight into the morphogenesis and function of the companion layer in hair folliclesLi Hong Gu
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
J Invest Dermatol 127:1061-73. 2007..These findings provide novel insight into the morphogenesis and properties of the Cl, and raise the distinct possibility that it is an integral part of the IRS compartment... - Loss of keratin 6 (K6) proteins reveals a function for intermediate filaments during wound repairPauline Wong
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
J Cell Biol 163:327-37. 2003.... - Keratin function in skin epithelia: a broadening palette with surprising shadesLi Hong Gu
Department of Biological Chemistry, Johns Hopkins University School of Medicine, 725 N Wolfe St, Baltimore, MD 21205, USA
Curr Opin Cell Biol 19:13-23. 2007..Other studies extended the role of keratin proteins in regulating the response to pro-apoptotic signals, and revealed their ability to modulate protein synthesis and cell size in epithelial cells challenged to grow... - Skin: an ideal model system to study keratin genes and proteinsKelsie M Bernot
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Methods Cell Biol 78:453-87. 2004 - A wound-induced keratin inhibits Src activity during keratinocyte migration and tissue repairJeremy D Rotty
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21202, USA
J Cell Biol 197:381-9. 2012..We conclude that K6 negatively regulates Src kinase activity and the migratory potential of skin keratinocytes during wound repair. Our findings may also be important in related contexts such as cancer... - Differential modulation of keratin expression by sulforaphane occurs via Nrf2-dependent and -independent pathways in skin epitheliaMichelle Kerns
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA
Mol Biol Cell 21:4068-75. 2010.... - Type II epithelial keratin 6hf (K6hf) is expressed in the companion layer, matrix, and medulla in anagen-stage hair folliclesZhiling Wang
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
J Invest Dermatol 121:1276-82. 2003..They also have obvious implications for the epithelial alterations associated with defects in keratin 6 genes... - Reprogramming of keratin biosynthesis by sulforaphane restores skin integrity in epidermolysis bullosa simplexMichelle L Kerns
Department of Biological Chemistry, 725 North Wolfe Street, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Proc Natl Acad Sci U S A 104:14460-5. 2007..Nrf2 induction is brought about by treatment with sulforaphane, a natural product. Sulforaphane thus represents an attractive option for the prevention of skin blistering associated with K14 mutations in EBS... - Keratin 17 modulates hair follicle cycling in a TNFalpha-dependent fashionXuemei Tong
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Genes Dev 20:1353-64. 2006..These findings identify K17 and TNFalpha as two novel and interdependent regulators of hair cycling... - A small surface hydrophobic stripe in the coiled-coil domain of type I keratins mediates tetramer stabilityKelsie M Bernot
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
J Cell Biol 168:965-74. 2005.... - Overexpressed transient receptor potential vanilloid 3 ion channels in skin keratinocytes modulate pain sensitivity via prostaglandin E2Susan M Huang
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21025, USA
J Neurosci 28:13727-37. 2008..Our results reveal a previously undescribed mechanism for keratinocyte participation in thermal pain transduction through keratinocyte TRPV3 ion channels and the intercellular messenger PGE(2)... - Intermediate filament scaffolds fulfill mechanical, organizational, and signaling functions in the cytoplasmSeyun Kim
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Genes Dev 21:1581-97. 2007..As this discovery process continues to unfold, a rationale for the large size of this family and the context-dependent regulation of its members is finally emerging... - Great promises yet to be fulfilled: defining keratin intermediate filament function in vivoPierre A Coulombe
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Eur J Cell Biol 83:735-46. 2004..Still, detailed studies of the phenotype exhibited by K6 and K17 null mice yielded novel insight into the properties and function of keratin IFs in vivo... - A keratin cytoskeletal protein regulates protein synthesis and epithelial cell growthSeyun Kim
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Nature 441:362-5. 2006..These findings reveal a new and unexpected role for the intermediate filament cytoskeleton in influencing cell growth and size by regulating protein synthesis... - A novel mouse type I intermediate filament gene, keratin 17n (K17n), exhibits preferred expression in nail tissueXuemei Tong
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
J Invest Dermatol 122:965-70. 2004..Depending on the existence of a human ortholog or a functional equivalent, our findings may also provide a molecular explanation for several unusual aspects of hK17-based diseases... - Hedgehog signaling, keratin 6 induction, and sebaceous gland morphogenesis: implications for pachyonychia congenita and related conditionsLi Hong Gu
Department of Biological Chemistry, Johns Hopkins University School of Medicine, 725 N Wolfe St, Baltimore, MD 21205, USA
Am J Pathol 173:752-61. 2008.... - Role for keratins 6 and 17 during wound closure in embryonic mouse skinStacy Mazzalupo
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Dev Dyn 226:356-65. 2003.... - Interaction between the keratin cytoskeleton and eEF1Bgamma affects protein synthesis in epithelial cellsSeyun Kim
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, 725 N Wolfe Street, Baltimore, Maryland 21205, USA
Nat Struct Mol Biol 14:982-3. 2007..These findings extend the emerging relationship between keratin proteins and the translational machinery... - Type I keratin 17 protein is phosphorylated on serine 44 by p90 ribosomal protein S6 kinase 1 (RSK1) in a growth- and stress-dependent fashionXiaoou Pan
Department of Biochemistry and Molecular Biology, Johns Hopkins University, Baltimore, Maryland 21205, USA
J Biol Chem 286:42403-13. 2011..These findings confirm and expand the tight link that has emerged between K17 up-regulation and growth and stress responses in the skin epithelium... - Self-organization of keratin intermediate filaments into cross-linked networksChang Hun Lee
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA
J Cell Biol 186:409-21. 2009..We propose that self-organization is a key determinant of the structural support function of keratin IFs in vivo... 
Structural basis for heteromeric assembly and perinuclear organization of keratin filamentsChang Hun Lee
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA
Nat Struct Mol Biol 19:707-15. 2012..We show that K14-Cys367 impacts nuclear shape in cultured keratinocytes and that mouse epidermal keratinocytes lacking K14 show aberrations in nuclear structure, highlighting a new function for keratin filaments...- Contribution of olfactory neural stem cells to tissue maintenance and regenerationCheuk T Leung
Center for Sensory Biology and the Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, 725 N Wolfe Street, PCTB 801, Baltimore, Maryland 21205, USA
Nat Neurosci 10:720-6. 2007..Our data support a new model of adult neurogenesis in which distinct cell populations mediate normal neuronal turnover and neuronal replacement upon traumatic injury... - Emerging role for the cytoskeleton as an organizer and regulator of translationSeyun Kim
The Solomon H Snyder Department of Neuroscience, School of Medicine, Johns Hopkins University, Baltimore, Maryland 21205, USA
Nat Rev Mol Cell Biol 11:75-81. 2010.... - Identification of novel interaction between annexin A2 and keratin 17: evidence for reciprocal regulationByung Min Chung
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland 21205, USA
J Biol Chem 287:7573-81. 2012..Down-regulation of K17 expression, in turn, results in decreased AnxA2 phosphorylation at Tyr-23. These findings uncover a novel interaction involving K17 and AnxA2 and identify AnxA2 as a potential regulator of keratin filaments... - Keratin 16-null mice develop palmoplantar keratoderma, a hallmark feature of pachyonychia congenita and related disordersJuliane C Lessard
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland 21205, USA
J Invest Dermatol 132:1384-91. 2012..Our findings call into question the view that PC-related PPK arises exclusively as a gain-of-function on account of dominantly acting mutated keratins, and highlight the key role of modifiers in the clinical heterogeneity of PC symptoms... - Intermediate filaments and tissue repairDaryle DePianto
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Exp Cell Res 301:68-76. 2004..Here we review the literature showing that modulation of cytoplasmic IFs occurs rapidly after injury to a variety of tissues and that such changes play an important role in the context of a timely repair response... - Cytoplasmic intermediate filaments revealed as dynamic and multipurpose scaffoldsPierre A Coulombe
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Nat Cell Biol 6:699-706. 2004.... - Modeling the self-organization property of keratin intermediate filamentsJin Seob Kim
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA
Biophys J 99:2748-56. 2010.... - Wound epithelialization: accelerating the pace of discoveryPierre A Coulombe
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
J Invest Dermatol 121:219-30. 2003 - Keratin 16 expression defines a subset of epithelial cells during skin morphogenesis and the hair cycleKelsie M Bernot
Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
J Invest Dermatol 119:1137-49. 2002.... - Mouse models of alopecia: identifying structural genes that are baldly neededXuemei Tong
Dept of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Trends Mol Med 9:79-84. 2003..Here we discuss transgenic mouse models that exhibit alopecia as a primary phenotype, resulting from the inactivation of genes encoding structural proteins... - Mathematical modeling of the impact of actin and keratin filaments on keratinocyte cell spreadingJin Seob Kim
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA
Biophys J 103:1828-38. 2012..These in silico findings provide further support for the notion that differentiation-related changes in the density and intracellular organization of keratin IFs affect tissue architecture in epidermis and related stratified epithelia... - Keratin 17 null mice exhibit age- and strain-dependent alopeciaKevin M McGowan
Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Genes Dev 16:1412-22. 2002.... - The mechanical properties of simple epithelial keratins 8 and 18: discriminating between interfacial and bulk elasticitiesSoichiro Yamada
Department of Chemical and Biomolecular Engineering, The Johns Hopkins University, 3400 North Charles Street, Baltimore, MD 21218, USA
J Struct Biol 143:45-55. 2003..The potential for modulation of mechanical properties through self-organization may be a general property of keratin polymers and contribute to their organization and function in vivo... - Keratins and protein synthesis: the plot thickensJuliane C Kellner
Department of Biochemistry and Molecular Biology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD 20723, USA
J Cell Biol 187:157-9. 2009..2009. J. Cell Biol. doi:10.1083/jcb.200906094) expand our understanding of how keratin proteins participate in the regulation of protein synthesis through their analysis of mice lacking the entire type II keratin gene cluster...