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Items: 1 to 50 of 74

1.

Pachyonychia Congenita.

Smith FJD, Hansen CD, Hull PR, Kaspar RL, McLean WHI, O’Toole E, Sprecher E.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2020.
2006 Jan 27 [updated 2017 Nov 30].

2.

Report of the 13th Annual International Pachyonychia Congenita Consortium Symposium.

Rittié L, Kaspar RL, Sprecher E, Smith FJD.

Br J Dermatol. 2017 May;176(5):1144-1147. doi: 10.1111/bjd.15417. Epub 2017 Mar 27.

3.

Oxidative stress and dysfunctional NRF2 underlie pachyonychia congenita phenotypes.

Kerns ML, Hakim JM, Lu RG, Guo Y, Berroth A, Kaspar RL, Coulombe PA.

J Clin Invest. 2016 Jun 1;126(6):2356-66. doi: 10.1172/JCI84870. Epub 2016 May 16.

4.

Imaging Functional Nucleic Acid Delivery to Skin.

Kaspar RL, Hickerson RP, González-González E, Flores MA, Speaker TP, Rogers FA, Milstone LM, Contag CH.

Methods Mol Biol. 2016;1372:1-24. doi: 10.1007/978-1-4939-3148-4_1.

PMID:
26530911
5.

Non-Invasive Intravital Imaging of siRNA-Mediated Mutant Keratin Gene Repression in Skin.

Hickerson RP, Speaker TJ, Lara MF, González-González E, Flores MA, Contag CH, Kaspar RL.

Mol Imaging Biol. 2016 Feb;18(1):34-42. doi: 10.1007/s11307-015-0875-z.

PMID:
26169581
6.

Differential fates of biomolecules delivered to target cells via extracellular vesicles.

Kanada M, Bachmann MH, Hardy JW, Frimannson DO, Bronsart L, Wang A, Sylvester MD, Schmidt TL, Kaspar RL, Butte MJ, Matin AC, Contag CH.

Proc Natl Acad Sci U S A. 2015 Mar 24;112(12):E1433-42. doi: 10.1073/pnas.1418401112. Epub 2015 Feb 23.

7.

Gene expression profiling in pachyonychia congenita skin.

Cao YA, Hickerson RP, Seegmiller BL, Grapov D, Gross MM, Bessette MR, Phinney BS, Flores MA, Speaker TJ, Vermeulen A, Bravo AA, Bruckner AL, Milstone LM, Schwartz ME, Rice RH, Kaspar RL.

J Dermatol Sci. 2015 Mar;77(3):156-65. doi: 10.1016/j.jdermsci.2015.01.001. Epub 2015 Jan 14.

8.

Pachyonychia congenita cornered: report on the 11th Annual International Pachyonychia Congenita Consortium Meeting.

O'Toole EA, Kaspar RL, Sprecher E, Schwartz ME, Rittié L.

Br J Dermatol. 2014 Nov;171(5):974-7. doi: 10.1111/bjd.13341. Epub 2014 Oct 15.

9.

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.

J Invest Dermatol. 2014 Mar;134(3):588-591. doi: 10.1038/jid.2013.392. No abstract available.

10.

Keratin 16 regulates innate immunity in response to epidermal barrier breach.

Lessard JC, Piña-Paz S, Rotty JD, Hickerson RP, Kaspar RL, Balmain A, Coulombe PA.

Proc Natl Acad Sci U S A. 2013 Nov 26;110(48):19537-42. doi: 10.1073/pnas.1309576110. Epub 2013 Nov 11.

11.

Gene Silencing in Skin After Deposition of Self-Delivery siRNA With a Motorized Microneedle Array Device.

Hickerson RP, Wey WC, Rimm DL, Speaker T, Suh S, Flores MA, Gonzalez-Gonzalez E, Leake D, Contag CH, Kaspar RL.

Mol Ther Nucleic Acids. 2013 Oct 22;2:e129. doi: 10.1038/mtna.2013.56.

12.

Gene silencing following siRNA delivery to skin via coated steel microneedles: In vitro and in vivo proof-of-concept.

Chong RH, Gonzalez-Gonzalez E, Lara MF, Speaker TJ, Contag CH, Kaspar RL, Coulman SA, Hargest R, Birchall JC.

J Control Release. 2013 Mar 28;166(3):211-9. doi: 10.1016/j.jconrel.2012.12.030. Epub 2013 Jan 8.

13.

Intravital fluorescence imaging of small interfering RNA-mediated gene repression in a dual reporter melanoma xenograft model.

Hickerson RP, Gonzalez-Gonzalez E, Vlassov AV, Li M, Lara MF, Contag CH, Kaspar RL.

Nucleic Acid Ther. 2012 Dec;22(6):438-43. doi: 10.1089/nat.2012.0364. Epub 2012 Oct 25.

14.

Designed guanidinium-rich amphipathic oligocarbonate molecular transporters complex, deliver and release siRNA in cells.

Geihe EI, Cooley CB, Simon JR, Kiesewetter MK, Edward JA, Hickerson RP, Kaspar RL, Hedrick JL, Waymouth RM, Wender PA.

Proc Natl Acad Sci U S A. 2012 Aug 14;109(33):13171-6. doi: 10.1073/pnas.1211361109. Epub 2012 Jul 30.

15.

Inhibition 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.

Hum Gene Ther. 2012 Aug;23(8):816-23. doi: 10.1089/hum.2011.211. Epub 2012 Jun 5.

16.

Generic and personalized RNAi-based therapeutics for a dominant-negative epidermal fragility disorder.

Leslie Pedrioli DM, Fu DJ, Gonzalez-Gonzalez E, Contag CH, Kaspar RL, Smith FJ, McLean WH.

J Invest Dermatol. 2012 Jun;132(6):1627-35. doi: 10.1038/jid.2012.28. Epub 2012 Mar 8.

17.

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.

Sci Rep. 2011;1:158. doi: 10.1038/srep00158. Epub 2011 Nov 15.

18.

In vivo sustained release of siRNA from solid lipid nanoparticles.

Lobovkina T, Jacobson GB, Gonzalez-Gonzalez E, Hickerson RP, Leake D, Kaspar RL, Contag CH, Zare RN.

ACS Nano. 2011 Dec 27;5(12):9977-83. doi: 10.1021/nn203745n. Epub 2011 Nov 18.

19.

An appraisal of oral retinoids in the treatment of pachyonychia congenita.

Gruber R, Edlinger M, Kaspar RL, Hansen CD, Leachman S, Milstone LM, Smith FJ, Sidoroff A, Fritsch PO, Schmuth M.

J Am Acad Dermatol. 2012 Jun;66(6):e193-9. doi: 10.1016/j.jaad.2011.02.003. Epub 2011 May 24.

PMID:
21601946
20.

Toward a treatment for pachyonychia congenita: report on the 7th Annual International Pachyonychia Congenita Consortium meeting.

Kaspar RL, Leachman SA, McLean WH, Schwartz ME.

J Invest Dermatol. 2011 May;131(5):1011-4. doi: 10.1038/jid.2011.44. No abstract available.

21.

Use of self-delivery siRNAs to inhibit gene expression in an organotypic pachyonychia congenita model.

Hickerson RP, Flores MA, Leake D, Lara MF, Contag CH, Leachman SA, Kaspar RL.

J Invest Dermatol. 2011 May;131(5):1037-44. doi: 10.1038/jid.2010.426. Epub 2011 Jan 20.

22.

In vivo imaging of human and mouse skin with a handheld dual-axis confocal fluorescence microscope.

Ra H, Piyawattanametha W, Gonzalez-Gonzalez E, Mandella MJ, Kino GS, Solgaard O, Leake D, Kaspar RL, Oro A, Contag CH.

J Invest Dermatol. 2011 May;131(5):1061-6. doi: 10.1038/jid.2010.401. Epub 2010 Dec 30.

23.

Development of quantitative molecular clinical end points for siRNA clinical trials.

Hickerson RP, Leachman SA, Pho LN, Gonzalez-Gonzalez E, Smith FJ, McLean WH, Contag CH, Leake D, Milstone LM, Kaspar RL.

J Invest Dermatol. 2011 May;131(5):1029-36. doi: 10.1038/jid.2010.372. Epub 2010 Dec 30.

24.

Development of skin-humanized mouse models of pachyonychia congenita.

García M, Larcher F, Hickerson RP, Baselga E, Leachman SA, Kaspar RL, Del Rio M.

J Invest Dermatol. 2011 May;131(5):1053-60. doi: 10.1038/jid.2010.353. Epub 2010 Dec 9.

25.

Biodegradable nanoparticles with sustained release of functional siRNA in skin.

Jacobson GB, Gonzalez-Gonzalez E, Spitler R, Shinde R, Leake D, Kaspar RL, Contag CH, Zare RN.

J Pharm Sci. 2010 Oct;99(10):4261-6. doi: 10.1002/jps.22147.

PMID:
20737633
26.

Assessing delivery and quantifying efficacy of small interfering ribonucleic acid therapeutics in the skin using a dual-axis confocal microscope.

Ra H, Gonzalez-Gonzalez E, Smith BR, Gambhir SS, Kino GS, Solgaard O, Kaspar RL, Contag CH.

J Biomed Opt. 2010 May-Jun;15(3):036027. doi: 10.1117/1.3432627.

27.

Silencing of reporter gene expression in skin using siRNAs and expression of plasmid DNA delivered by a soluble protrusion array device (PAD).

Gonzalez-Gonzalez E, Speaker TJ, Hickerson RP, Spitler R, Flores MA, Leake D, Contag CH, Kaspar RL.

Mol Ther. 2010 Sep;18(9):1667-74. doi: 10.1038/mt.2010.126. Epub 2010 Jun 22.

28.

Increased interstitial pressure improves nucleic acid delivery to skin enabling a comparative analysis of constitutive promoters.

González-González E, Ra H, Spitler R, Hickerson RP, Contag CH, Kaspar RL.

Gene Ther. 2010 Oct;17(10):1270-8. doi: 10.1038/gt.2010.74. Epub 2010 May 13.

PMID:
20463756
29.

Nanoparticle formation of organic compounds with retained biological activity.

Jacobson GB, Shinde R, McCullough RL, Cheng NJ, Creasman A, Beyene A, Hickerson RP, Quan C, Turner C, Kaspar RL, Contag CH, Zare RN.

J Pharm Sci. 2010 Jun;99(6):2750-5. doi: 10.1002/jps.22035.

PMID:
20039390
30.

First-in-human mutation-targeted siRNA phase Ib trial of an inherited skin disorder.

Leachman SA, Hickerson RP, Schwartz ME, Bullough EE, Hutcherson SL, Boucher KM, Hansen CD, Eliason MJ, Srivatsa GS, Kornbrust DJ, Smith FJ, McLean WI, Milstone LM, Kaspar RL.

Mol Ther. 2010 Feb;18(2):442-6. doi: 10.1038/mt.2009.273. Epub 2009 Nov 24.

31.

Achieving successful delivery of nucleic acids to skin: 6th Annual Meeting of the International Pachyonychia Congenita Consortium.

Kaspar RL, McLean WH, Schwartz ME.

J Invest Dermatol. 2009 Sep;129(9):2085-7. doi: 10.1038/jid.2009.220. No abstract available.

32.

Association of bacterial colonization at the time of presentation to a combat support hospital in a combat zone with subsequent 30-day colonization or infection.

Kaspar RL, Griffith ME, Mann PB, Lehman DJ, Conger NG, Hospenthal DR, Murray CK.

Mil Med. 2009 Sep;174(9):899-903.

PMID:
19780364
33.

Rapamycin selectively inhibits expression of an inducible keratin (K6a) in human keratinocytes and improves symptoms in pachyonychia congenita patients.

Hickerson RP, Leake D, Pho LN, Leachman SA, Kaspar RL.

J Dermatol Sci. 2009 Nov;56(2):82-8. doi: 10.1016/j.jdermsci.2009.07.008. Epub 2009 Aug 21.

PMID:
19699613
34.

Therapeutic silencing of an endogenous gene by siRNA cream in an arthritis model mouse.

Takanashi M, Oikawa K, Sudo K, Tanaka M, Fujita K, Ishikawa A, Nakae S, Kaspar RL, Matsuzaki M, Kudo M, Kuroda M.

Gene Ther. 2009 Aug;16(8):982-9. doi: 10.1038/gt.2009.66. Epub 2009 May 28.

PMID:
19474812
35.

siRNA silencing of keratinocyte-specific GFP expression in a transgenic mouse skin model.

Gonzalez-Gonzalez E, Ra H, Hickerson RP, Wang Q, Piyawattanametha W, Mandella MJ, Kino GS, Leake D, Avilion AA, Solgaard O, Doyle TC, Contag CH, Kaspar RL.

Gene Ther. 2009 Aug;16(8):963-72. doi: 10.1038/gt.2009.62. Epub 2009 May 28.

PMID:
19474811
36.

Hairpin ribozyme-antisense RNA constructs can act as molecular Lassos.

Dallas A, Balatskaya SV, Kuo TC, Ilves H, Vlassov AV, Kaspar RL, Kisich KO, Kazakov SA, Johnston BH.

Nucleic Acids Res. 2008 Dec;36(21):6752-66. doi: 10.1093/nar/gkn637. Epub 2008 Oct 25.

37.

Stability study of unmodified siRNA and relevance to clinical use.

Hickerson RP, Vlassov AV, Wang Q, Leake D, Ilves H, Gonzalez-Gonzalez E, Contag CH, Johnston BH, Kaspar RL.

Oligonucleotides. 2008 Dec;18(4):345-54. doi: 10.1089/oli.2008.0149.

38.

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.

J Dermatol Sci. 2008 Sep;51(3):151-7. doi: 10.1016/j.jdermsci.2008.04.003. Epub 2008 May 20. Review.

39.

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.

J Invest Dermatol. 2008 Mar;128(3):594-605. Epub 2007 Oct 11.

40.

Development of therapeutic siRNAs for pachyonychia congenita.

Smith FJ, Hickerson RP, Sayers JM, Reeves RE, Contag CH, Leake D, Kaspar RL, McLean WH.

J Invest Dermatol. 2008 Jan;128(1):50-8. Epub 2007 Aug 30.

41.

shRNAs targeting hepatitis C: effects of sequence and structural features, and comparision with siRNA.

Vlassov AV, Korba B, Farrar K, Mukerjee S, Seyhan AA, Ilves H, Kaspar RL, Leake D, Kazakov SA, Johnston BH.

Oligonucleotides. 2007 Summer;17(2):223-36.

PMID:
17638526
42.

Identification of AUF1 as a rapamycin-responsive binding protein to the 5'-terminal oligopyrimidine element of mRNAs.

Kakegawa T, Ohuchi N, Hayakawa A, Hirata S, Matsuda M, Kogure K, Kobayashi H, Inoue A, Kaspar RL.

Arch Biochem Biophys. 2007 Sep 1;465(1):274-81. Epub 2007 Jun 9.

PMID:
17603996
43.

Delivery and inhibition of reporter genes by small interfering RNAs in a mouse skin model.

Wang Q, Ilves H, Chu P, Contag CH, Leake D, Johnston BH, Kaspar RL.

J Invest Dermatol. 2007 Nov;127(11):2577-84. Epub 2007 May 24.

44.

SiRNA-mediated selective inhibition of mutant keratin mRNAs responsible for the skin disorder pachyonychia congenita.

Hickerson RP, Smith FJ, McLean WH, Landthaler M, Leube RE, Kaspar RL.

Ann N Y Acad Sci. 2006 Oct;1082:56-61.

PMID:
17145926
45.

Inhibition of hepatitis C IRES-mediated gene expression by small hairpin RNAs in human hepatocytes and mice.

Ilves H, Kaspar RL, Wang Q, Seyhan AA, Vlassov AV, Contag CH, Leake D, Johnston BH.

Ann N Y Acad Sci. 2006 Oct;1082:52-5.

PMID:
17145925
46.

Challenges in developing therapies for rare diseases including pachyonychia congenita.

Kaspar RL.

J Investig Dermatol Symp Proc. 2005 Oct;10(1):62-6. Review.

47.

Clinical and pathological features of pachyonychia congenita.

Leachman SA, Kaspar RL, Fleckman P, Florell SR, Smith FJ, McLean WH, Lunny DP, Milstone LM, van Steensel MA, Munro CS, O'Toole EA, Celebi JT, Kansky A, Lane EB.

J Investig Dermatol Symp Proc. 2005 Oct;10(1):3-17. Review.

48.
49.

Complete, gene-specific siRNA libraries: production and expression in mammalian cells.

Seyhan AA, Vlassov AV, Ilves H, Egry L, Kaspar RL, Kazakov SA, Johnston BH.

RNA. 2005 May;11(5):837-46.

50.

Differential translation of TOP mRNAs in rapamycin-treated human B lymphocytes.

Zhu J, Spencer ED, Kaspar RL.

Biochim Biophys Acta. 2003 Jul 9;1628(1):50-5.

PMID:
12850272

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