2016
Connexin channels in congenital skin disorders
Lilly E, Sellitto C, Milstone LM, White TW. Connexin channels in congenital skin disorders. Seminars In Cell And Developmental Biology 2016, 50: 4-12. PMID: 26775130, PMCID: PMC4779425, DOI: 10.1016/j.semcdb.2015.11.018.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsConnexin mutationsDifferent connexin genesSubtle functional differencesConnexin functionConnexin genesDominant gainConnexin channelsHeteromeric hemichannelsSpectrum of phenotypesFunctional roleConnexin 26 mutationsFunctional differencesCongenital skin disorderMutationsGap junctionsHemichannelsCalcium regulationConnexinsConnexin 43Detailed understandingCalcium permeabilitySkin disordersPalmoplantar keratodermaDeafness syndromeGenesImaging Functional Nucleic Acid Delivery to Skin
Kaspar RL, Hickerson RP, González-González E, Flores MA, Speaker TP, Rogers FA, Milstone LM, Contag CH. Imaging Functional Nucleic Acid Delivery to Skin. Methods In Molecular Biology 2016, 1372: 1-24. PMID: 26530911, DOI: 10.1007/978-1-4939-3148-4_1.Peer-Reviewed Original ResearchConceptsNucleic acid deliveryNucleic acid-based therapiesAcid deliveryAcid-based therapiesFunctional deliveryFunctional nucleic acid deliveryTherapeutic nucleic acidsNucleic acidsOptical imaging technologiesReal-time monitoringTriplex nucleic acidsInteraction of lightMonogenic skin diseasesLight-tissue interactionsGene editingGene silencingOptical imagingNoninvasive imaging technologyTopical deliveryTime monitoringImaging technologyReporter geneLayered structureGeneral strategyEnzymatic reactions
2015
The Protein Acyl Transferase ZDHHC21 Modulates &agr;1 Adrenergic Receptor Function and Regulates Hemodynamics
Marin EP, Jozsef L, Di Lorenzo A, Held KF, Luciano AK, Melendez J, Milstone LM, Velazquez H, Sessa WC. The Protein Acyl Transferase ZDHHC21 Modulates &agr;1 Adrenergic Receptor Function and Regulates Hemodynamics. Arteriosclerosis Thrombosis And Vascular Biology 2015, 36: 370-379. PMID: 26715683, PMCID: PMC4984414, DOI: 10.1161/atvbaha.115.306942.Peer-Reviewed Original ResearchMeSH KeywordsAcyltransferasesAdrenergic alpha-1 Receptor AgonistsAnimalsAortaBlood PressureDose-Response Relationship, DrugEpinephrineFibroblastsGenotypeHeart RateHEK293 CellsHemodynamicsHumansHypotensionLipoylationMesenteric ArteriesMice, Inbred C57BLMice, Mutant StrainsMutationNorepinephrinePhenotypePhenylephrineReceptors, Adrenergic, alpha-1Signal TransductionTachycardiaTime FactorsTransfectionVasoconstrictionConceptsProtein acyl transferasesNovel molecular modeZDHHC familyLipid palmitateZDHHC enzymesVascular toneNumerous proteinsPossible molecular mechanismsProtein localizationAdrenergic receptor gene expressionGene expressionMolecular mechanismsVascular functionBiochemical studiesReceptor gene expressionAcyl transferaseMolecular modePalmitoylationΑ1-adrenergic receptor agonistTelemetry studiesInfusion of phenylephrinePhysiological studiesΑ1-adrenergic receptorsReceptor functionAdrenergic receptor agonist
2014
Report of the 10th Annual International Pachyonychia Congenita Consortium Meeting
van Steensel MAM, Coulombe PA, Kaspar RL, Milstone LM, McLean IWH, Roop DR, Smith FJD, Sprecher E, Schwartz ME. Report of the 10th Annual International Pachyonychia Congenita Consortium Meeting. Journal Of Investigative Dermatology 2014, 134: 588-591. PMID: 24518109, PMCID: PMC3930927, DOI: 10.1038/jid.2013.392.Commentaries, Editorials and Letters
2012
Local Delivery of Gene-Modifying Triplex-Forming Molecules to the Epidermis
Rogers FA, Hu RH, Milstone LM. Local Delivery of Gene-Modifying Triplex-Forming Molecules to the Epidermis. Journal Of Investigative Dermatology 2012, 133: 685-691. PMID: 23014335, PMCID: PMC3532560, DOI: 10.1038/jid.2012.351.Peer-Reviewed Original ResearchConceptsIntradermal deliveryHairless miceTransgenic miceBasal keratinocytesMethod of deliveryNuclei of keratinocytesIntraperitoneal administrationSitu gene correctionBack skinLocal deliveryMiceDisease-causing genesEpidermal keratinocytesKeratinocytesLocal administrationAdministrationDeliverySkinEpidermisDaysInhibition of CD44 Gene Expression in Human Skin Models, Using Self-Delivery Short Interfering RNA Administered by Dissolvable Microneedle Arrays
Lara MF, González-González E, Speaker TJ, Hickerson RP, Leake D, Milstone LM, Contag CH, Kaspar RL. Inhibition of CD44 Gene Expression in Human Skin Models, Using Self-Delivery Short Interfering RNA Administered by Dissolvable Microneedle Arrays. Human Gene Therapy 2012, 23: 816-823. PMID: 22480249, PMCID: PMC3413896, DOI: 10.1089/hum.2011.211.Peer-Reviewed Original ResearchConceptsTransgenic mouse modelInhibition of CD44CD44-specific antibodiesCD44 mRNA levelsDissolvable microneedle arraysGene expressionMouse modelXenograft modelSkin disordersCD44 gene expressionHuman skin modelTarget cellsMRNA levelsHuman skin equivalentsKeratinocyte membraneTreatmentInhibitionStratum corneumTarget gene expressionSiRNASkin equivalentsEpidermal equivalentsMicroneedle arraysSkin modelExpressionImpact of epidermal desquamation on tissue stores of iron
Milstone LM, Hu RH, Dziura JD, Zhou J. Impact of epidermal desquamation on tissue stores of iron. Journal Of Dermatological Science 2012, 67: 9-14. PMID: 22575277, PMCID: PMC3374011, DOI: 10.1016/j.jdermsci.2012.04.003.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAntimicrobial Cationic PeptidesCell ProliferationDisease Models, AnimalEpidermisFemaleGene Expression RegulationHemochromatosisHemochromatosis ProteinHepcidinsHistocompatibility Antigens Class IHomeostasisHuman papillomavirus 16HumansIronKidneyLiverMaleMembrane ProteinsMiceMice, 129 StrainMice, Inbred C57BLMice, KnockoutMice, TransgenicPapillomavirus InfectionsReceptors, TransferrinConceptsIron metabolismDifferent mouse modelsSignificant reductionHPV16 E7 geneSystemic iron metabolismSystemic proteinsIron statusMouse modelTissue storesE7 genesSystemic levelsEpidermal turnoverInternal organsEpidermal desquamationTransferrin receptorMetabolic studiesKnockout modelsCorneocyte desquamationDesquamationKidneyHuman skinEpidermal homeostasisMetabolismEpidermisSkin
2011
Visualization of plasmid delivery to keratinocytes in mouse and human epidermis
González-González E, Kim YC, Speaker TJ, Hickerson RP, Spitler R, Birchall JC, Lara MF, Hu RH, Liang Y, Kirkiles-Smith N, Prausnitz MR, Milstone LM, Contag CH, Kaspar RL. Visualization of plasmid delivery to keratinocytes in mouse and human epidermis. Scientific Reports 2011, 1: 158. PMID: 22355673, PMCID: PMC3240989, DOI: 10.1038/srep00158.Peer-Reviewed Original ResearchConceptsIdeal target organHuman skin xenograftsAccessibility of skinSkin delivery technologiesNon-invasive intravital imagingIntradermal injectionSkin xenograftsClinical utilityTarget organsPatient-friendly deliveryMouse skinBioluminescence imagingSkin model systemIntravital imagingNoninvasive assayTransfection of cellsIntravital fluorescenceHuman epidermisIndividual keratinocytesKeratinocytesSkinNucleic acid-based therapeuticsMiceReporter plasmidDelivery technologies
2008
Therapeutic siRNAs for dominant genetic skin disorders including pachyonychia congenita
Leachman SA, Hickerson RP, Hull PR, Smith FJ, Milstone LM, Lane EB, Bale SJ, Roop DR, McLean WH, Kaspar RL. Therapeutic siRNAs for dominant genetic skin disorders including pachyonychia congenita. Journal Of Dermatological Science 2008, 51: 151-157. PMID: 18495438, PMCID: PMC2587483, DOI: 10.1016/j.jdermsci.2008.04.003.Peer-Reviewed Original ResearchConceptsPachyonychia congenitaQuantitative RT-PCRRT-PCRReal-time RT-PCRQuantitative real-time RT-PCRClinical efficacyGenetic skin disordersClinical trialsCandidate siRNASkin disordersRNA interferenceNovel therapeuticsPotent inhibitionTherapeutic siRNAsClinical applicationDisordersGenetic disordersPhysiciansCongenitaMutant allelesSiRNAHuman diseasesKeratin disordersPatientsDisease
2007
Single-Nucleotide-Specific siRNA Targeting in a Dominant-Negative Skin Model
Hickerson RP, Smith FJ, Reeves RE, Contag CH, Leake D, Leachman SA, Milstone LM, McLean WH, Kaspar RL. Single-Nucleotide-Specific siRNA Targeting in a Dominant-Negative Skin Model. Journal Of Investigative Dermatology 2007, 128: 594-605. PMID: 17914454, DOI: 10.1038/sj.jid.5701060.Peer-Reviewed Original ResearchConceptsWild-type gene expressionBicistronic reporter constructFilament formationDominant-negative genetic disordersNumerous genetic disordersGenetic disordersKeratin filament formationRNA interferenceMutant formsReporter constructsGene expressionMutant mRNAFluorescent reportersMutant allelesCell culture modelSiRNAsSimultaneous expressionK6aK mutationPachyonychia congenitaExpressionCulture modelHuman keratinocytesSiRNASelective inhibition
2006
Stratum-Specific Expression of Human Transferrin Receptor Increases Iron in Mouse Epidermis
Milstone LM, Adams BD, Zhou J, Sanchez V, Shofner J. Stratum-Specific Expression of Human Transferrin Receptor Increases Iron in Mouse Epidermis. Journal Of Investigative Dermatology 2006, 126: 648-652. PMID: 16424878, PMCID: PMC2243218, DOI: 10.1038/sj.jid.5700127.Peer-Reviewed Original ResearchConceptsTransgenic miceStratum-specific expressionTransferrin receptorKeratin 14 promoterTransferrin receptor expressionBody iron lossesReceptor expressionHistological changesSite-dependent differencesLittermate controlsNormal epidermisReceptorsMiceMouse epidermisEpidermal desquamationFerritin expressionHuman transferrin receptorInvolucrin promoterKeratinocytesTransgenic animalsEpidermisAgeExtra ironExpressionAnimals
2005
Iron in Skin of Mice with Three Etiologies of Systemic Iron Overload
Adams BD, Lazova R, Andrews NC, Milstone LM. Iron in Skin of Mice with Three Etiologies of Systemic Iron Overload. Journal Of Investigative Dermatology 2005, 125: 1200-1205. PMID: 16354190, PMCID: PMC2243217, DOI: 10.1111/j.0022-202x.2005.23949.x.Peer-Reviewed Original ResearchConceptsSystemic iron overloadHigh-iron dietSkin ironIron overloadIron dietIron levelsSkin of miceTissue iron levelsCutaneous manifestationsSkin of individualsSkin toxicityHistological abnormalitiesHistological changesMouse modelParenteral injectionParenteral administrationHereditary hemochromatosisTissue toxicityHuman hemochromatosisMiceSkin histologyHemochromatosis geneHemochromatosisHigh dietDermisGene Therapy for Autosomal Dominant Disorders of Keratin
Lewin AS, Glazer PM, Milstone LM. Gene Therapy for Autosomal Dominant Disorders of Keratin. Journal Of Investigative Dermatology Symposium Proceedings 2005, 10: 47-61. PMID: 16250209, DOI: 10.1111/j.1087-0024.2005.10207.x.Commentaries, Editorials and LettersMeSH KeywordsAnimalsDarier DiseaseDependovirusEctodermal DysplasiaEpidermolysis Bullosa SimplexGene SilencingGene TargetingGenes, DominantGenetic TherapyGenetic VectorsHumansKeratinsKeratoderma, PalmoplantarMiceMutationNails, MalformedOligonucleotides, AntisenseRNA InterferenceRNA, CatalyticRNA, Small InterferingConceptsRNA knockdown approachGene correctionGene therapyViral vectorsEpidermal skin cellsKeratin diseasesKnockdown approachRNA interferenceGene expressionEpidermolysis bullosa simplexToxic proteinsDominant mutationsGenetic diseasesGenetic therapiesKeratin filamentsEpidermal diseasesGenetic defectsTissue cultureSelective inhibitorSkin cellsAttractive alternativeAutosomal dominant disorderRecent innovationsDominant disorderNear future
2004
Long-Term Murine Keratinocyte Cultures Become Tetraploid, Yet Maintain the Ability to Stratify
Sanchez V, Zhou J, LaCivita D, Milstone L. Long-Term Murine Keratinocyte Cultures Become Tetraploid, Yet Maintain the Ability to Stratify. Journal Of Investigative Dermatology 2004, 123: 403-404. PMID: 15245442, DOI: 10.1111/j.0022-202x.2004.23218.x.Peer-Reviewed Original Research
1994
Epican, a heparan/chondroitin sulfate proteoglycan form of CD44, mediates cell-cell adhesion
Milstone L, Hough-Monroe L, Kugelman L, Bender J, Haggerty J. Epican, a heparan/chondroitin sulfate proteoglycan form of CD44, mediates cell-cell adhesion. Journal Of Cell Science 1994, 107: 3183-3190. PMID: 7699015, DOI: 10.1242/jcs.107.11.3183.Peer-Reviewed Original ResearchOverexpression of parathyroid hormone-related protein in the skin of transgenic mice interferes with hair follicle development.
Wysolmerski JJ, Broadus AE, Zhou J, Fuchs E, Milstone LM, Philbrick WM. Overexpression of parathyroid hormone-related protein in the skin of transgenic mice interferes with hair follicle development. Proceedings Of The National Academy Of Sciences Of The United States Of America 1994, 91: 1133-1137. PMID: 7508121, PMCID: PMC521468, DOI: 10.1073/pnas.91.3.1133.Peer-Reviewed Original ResearchConceptsFollicle developmentTransgenic miceHair follicle developmentHuman keratin 14 promoterParathyroid hormone-related proteinNormal hair follicle developmentOverexpression of PTHrPHormone-related proteinHormone-related peptideKeratin 14 promoterHumoral hypercalcemiaPTHrP geneNormal tissuesMicePTHrPSkinOverexpressionCellular differentiationEarly stagesHypercalcemiaMalignancySyndromePeptidesFollicles
1992
Parathyroid hormone-related protein. Evidence for secretion of a novel mid-region fragment by three different cell types.
Soifer NE, Dee KE, Insogna KL, Burtis WJ, Matovcik LM, Wu TL, Milstone LM, Broadus AE, Philbrick WM, Stewart AF. Parathyroid hormone-related protein. Evidence for secretion of a novel mid-region fragment by three different cell types. Journal Of Biological Chemistry 1992, 267: 18236-18243. PMID: 1517251, DOI: 10.1016/s0021-9258(19)37178-9.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsCarcinoma, Renal CellCell LineElectrophoresis, Polyacrylamide GelFluorescent Antibody TechniqueHumansImmunohistochemistryKidney NeoplasmsKineticsMolecular Sequence DataParathyroid HormoneParathyroid Hormone-Related ProteinPeptide FragmentsProtein BiosynthesisProteinsTransfectionConceptsMid-region fragmentCell typesSecretory formBasic amino acid motifHormone-related proteinAmino acid motifsImportant biological functionsAmino acid sequenceExtensive post-translational processingPost-translational processingDifferent cell typesHuman renal carcinoma cellsInterspecies conservationSodium dodecyl sulfate-polyacrylamide gel electrophoresis analysisRenal carcinoma cellsAcid motifGel electrophoresis analysisAcid sequenceBiological functionsParathyroid hormone-related proteinRegion-specific immunoassaysRat insulinoma cellsCell extractsCleavage siteAmino acidsHyaluronidase from infective Ancylostoma hookworm larvae and its possible function as a virulence factor in tissue invasion and in cutaneous larva migrans
Hotez P, Narasimhan S, Haggerty J, Milstone L, Bhopale V, Schad G, Richards F. Hyaluronidase from infective Ancylostoma hookworm larvae and its possible function as a virulence factor in tissue invasion and in cutaneous larva migrans. Infection And Immunity 1992, 60: 1018-1023. PMID: 1541516, PMCID: PMC257588, DOI: 10.1128/iai.60.3.1018-1023.1992.Peer-Reviewed Original Research
1991
Immunoaffinity purification of parathyroid hormone‐related protein from bovine milk and human keratinocyte‐conditioned medium
Stewart A, Wu T, Insogna K, Milstone L, Burtis W. Immunoaffinity purification of parathyroid hormone‐related protein from bovine milk and human keratinocyte‐conditioned medium. Journal Of Bone And Mineral Research 1991, 6: 305-311. PMID: 2035357, DOI: 10.1002/jbmr.5650060313.Peer-Reviewed Original ResearchAntiviral Activity of 5-Iodo-2’-Deoxyuridine and Related Drugs in Human Keratinocytes Infected in vitro with Herpes simplex Virus Type 1
Reuveni H, Bull CO, Landry ML, Milstone LM, Schwartz PM. Antiviral Activity of 5-Iodo-2’-Deoxyuridine and Related Drugs in Human Keratinocytes Infected in vitro with Herpes simplex Virus Type 1. Skin Pharmacology And Physiology 1991, 4: 291-297. PMID: 1665069, DOI: 10.1159/000210964.Peer-Reviewed Original ResearchConceptsGuinea pig embryo cellsHerpes simplex virus type 1Simplex virus type 1Virus type 1Antiviral activityThymidine phosphorylaseHuman keratinocytesType 1Cutaneous viral infectionsTopical antiviral agentsAntiviral agentsViral infectionExperimental animalsEmbryo cellsRelated drugsIUdRInfected cellsKeratinocytesDrugsCellsActivityInfectionPhosphorylase