PubMed

AAV-Mediated Rhodopsin Replacement Provides Therapeutic Benefit in Mice with a Targeted Disruption of the Rhodopsin Gene.

Palfi A, Millington-Ward S, Chadderton N, O'Reilly M, Goldmann T, Humphries MM, Wolfrum U, Humphries P, Kenna PF, Farrar GJ.

Hum Gene Ther. 2009 Oct 13. [Epub ahead of print]PMID: 19824806 [PubMed - as supplied by publisher]Related articles

An experimental platform for systemic drug delivery to the retina.

Campbell M, Nguyen AT, Kiang AS, Tam LC, Gobbo OL, Kerskens C, Ni Dhubhghaill S, Humphries MM, Farrar GJ, Kenna PF, Humphries P.

Proc Natl Acad Sci U S A. 2009 Oct 20;106(42):17817-22. Epub 2009 Oct 12.PMID: 19822744 [PubMed - in process]Related articlesFree article

Study of gene targeted mouse models of splicing factor gene Prpf31 implicated in human autosomal dominant retinitis pigmentosa (RP).

Bujakowska KM, Maubaret C, Chakarova CF, Tanimoto N, Beck SC, Fahl E, Humphries MM, Kenna P, Makarov E, Makarova O, Paquet-Durand F, Ekström P, van Veen T, Leveillard T, Humphries P, Seeliger M, Bhattacharya SS.

Invest Ophthalmol Vis Sci. 2009 Jul 2. [Epub ahead of print]PMID: 19578015 [PubMed - as supplied by publisher]Related articles

Improved retinal function in a mouse model of dominant retinitis pigmentosa following AAV-delivered gene therapy.

Chadderton N, Millington-Ward S, Palfi A, O'Reilly M, Tuohy G, Humphries MM, Li T, Humphries P, Kenna PF, Farrar GJ.

Mol Ther. 2009 Apr;17(4):593-9. Epub 2009 Jan 27.PMID: 19174761 [PubMed - indexed for MEDLINE]Related articles

RNAi-mediated reversible opening of the blood-brain barrier.

Campbell M, Kiang AS, Kenna PF, Kerskens C, Blau C, O'Dwyer L, Tivnan A, Kelly JA, Brankin B, Farrar GJ, Humphries P.

J Gene Med. 2008 Aug;10(8):930-47.PMID: 18509865 [PubMed - indexed for MEDLINE]Related articles

Therapeutic benefit derived from RNAi-mediated ablation of IMPDH1 transcripts in a murine model of autosomal dominant retinitis pigmentosa (RP10).

Tam LC, Kiang AS, Kennan A, Kenna PF, Chadderton N, Ader M, Palfi A, Aherne A, Ayuso C, Campbell M, Reynolds A, McKee A, Humphries MM, Farrar GJ, Humphries P.

Hum Mol Genet. 2008 Jul 15;17(14):2084-100. Epub 2008 Apr 1.PMID: 18385099 [PubMed - indexed for MEDLINE]Related articlesFree article

Influence of a quantitative trait locus on mouse chromosome 19 to the light-adapted electroretinogram.

Reynolds AL, Danciger M, Farrar GJ, Humphries P, Kenna PF.

Invest Ophthalmol Vis Sci. 2008 Sep;49(9):4058-63. Epub 2008 Mar 14.PMID: 18344449 [PubMed - indexed for MEDLINE]Related articlesFree article

Retinal cells integrate into the outer nuclear layer and differentiate into mature photoreceptors after subretinal transplantation into adult mice.

Bartsch U, Oriyakhel W, Kenna PF, Linke S, Richard G, Petrowitz B, Humphries P, Farrar GJ, Ader M.

Exp Eye Res. 2008 Apr;86(4):691-700. Epub 2008 Feb 3.PMID: 18329018 [PubMed - indexed for MEDLINE]Related articles

Variation in the electroretinogram of C57BL/6 substrains of mouse.

Reynolds AL, Farrar GJ, Humphries P, Kenna PF.

Adv Exp Med Biol. 2008;613:383-91. No abstract available. PMID: 18188968 [PubMed - indexed for MEDLINE]Related articles

Reference gene selection for real-time rtPCR in human epidermal keratinocytes.

Allen D, Winters E, Kenna PF, Humphries P, Farrar GJ.

J Dermatol Sci. 2008 Mar;49(3):217-25. Epub 2007 Dec 3.PMID: 18061409 [PubMed - indexed for MEDLINE]Related articles

Altered retinal microRNA expression profile in a mouse model of retinitis pigmentosa.

Loscher CJ, Hokamp K, Kenna PF, Ivens AC, Humphries P, Palfi A, Farrar GJ.

Genome Biol. 2007;8(11):R248.PMID: 18034880 [PubMed - indexed for MEDLINE]Related articlesFree article

A transgenic mouse model for gene therapy of rhodopsin-linked Retinitis Pigmentosa.

O'Reilly M, Millington-Ward S, Palfi A, Chadderton N, Cronin T, McNally N, Humphries MM, Humphries P, Kenna PF, Farrar GJ.

Vision Res. 2008 Feb;48(3):386-91. Epub 2007 Oct 24.PMID: 17920651 [PubMed - indexed for MEDLINE]Related articles

Altered expression and interaction of adherens junction proteins in the developing OLM of the Rho(-/-) mouse.

Campbell M, Humphries M, Kenna P, Humphries P, Brankin B.

Exp Eye Res. 2007 Nov;85(5):714-20. Epub 2007 Aug 15.PMID: 17888904 [PubMed - indexed for MEDLINE]Related articles

RNA interference-mediated suppression and replacement of human rhodopsin in vivo.

O'Reilly M, Palfi A, Chadderton N, Millington-Ward S, Ader M, Cronin T, Tuohy T, Auricchio A, Hildinger M, Tivnan A, McNally N, Humphries MM, Kiang AS, Humphries P, Kenna PF, Farrar GJ.

Am J Hum Genet. 2007 Jul;81(1):127-35. Epub 2007 May 23.PMID: 17564969 [PubMed - indexed for MEDLINE]Related articlesFree article

Development of strategies for conditional RNA interference.

Allen D, Kenna PF, Palfi A, McMahon HP, Millington-Ward S, O'Reilly M, Humphries P, Farrar GJ.

J Gene Med. 2007 Apr;9(4):287-98.PMID: 17397092 [PubMed - indexed for MEDLINE]Related articles

Molecular mechanisms of photoreceptor degeneration in RP caused by IMPDH1 mutations.

Aherne A, Kennan A, Kenna PF, McNally N, Farrar GJ, Humphries P.

Adv Exp Med Biol. 2006;572:81-7. No abstract available. PMID: 17249559 [PubMed - indexed for MEDLINE]Related articles

Aberrant retinal tight junction and adherens junction protein expression in an animal model of autosomal dominant Retinitis pigmentosa: the Rho(-/-) mouse.

Campbell M, Humphries M, Kennan A, Kenna P, Humphries P, Brankin B.

Exp Eye Res. 2006 Sep;83(3):484-92. Epub 2006 Apr 27.PMID: 16643895 [PubMed - indexed for MEDLINE]Related articles

Comparison of a rule-based algorithm with a phenotype-based algorithm for the interpretation of HIV genotypes in guiding salvage regimens in HIV-infected patients by a randomized clinical trial: the mutations and salvage study.

Gianotti N, Mondino V, Rossi MC, Chiesa E, Mezzaroma I, Ladisa N, Guaraldi G, Torti C, Tarquini P, Castelli P, Di Carlo A, Boeri E, Keulen W, Kenna PM, Lazzarin A; Mutations and Salvage (MuSa) Study Group.

Clin Infect Dis. 2006 May 15;42(10):1470-80. Epub 2006 Apr 13.PMID: 16619162 [PubMed - indexed for MEDLINE]Related articles

RNAi-based suppression and replacement of rds-peripherin in retinal organotypic culture.

Palfi A, Ader M, Kiang AS, Millington-Ward S, Clark G, O'Reilly M, McMahon HP, Kenna PF, Humphries P, Farrar GJ.

Hum Mutat. 2006 Mar;27(3):260-8.PMID: 16419083 [PubMed - indexed for MEDLINE]Related articles

Toward a gene therapy for dominant disease: validation of an RNA interference-based mutation-independent approach.

Kiang AS, Palfi A, Ader M, Kenna PF, Millington-Ward S, Clark G, Kennan A, O'reilly M, Tam LC, Aherne A, McNally N, Humphries P, Farrar GJ.

Mol Ther. 2005 Sep;12(3):555-61.PMID: 15967729 [PubMed - indexed for MEDLINE]Related articles

RNAi of COL1A1 in mesenchymal progenitor cells.

Millington-Ward S, McMahon HP, Allen D, Tuohy G, Kiang AS, Palfi A, Kenna PF, Humphries P, Farrar GJ.

Eur J Hum Genet. 2004 Oct;12(10):864-6.PMID: 15241481 [PubMed - indexed for MEDLINE]Related articlesFree article

On the role of IMPDH1 in retinal degeneration.

Kennan A, Aherne A, Bowne SJ, Daiger SP, Farrar GJ, Kenna PF, Humphries P.

Adv Exp Med Biol. 2003;533:13-8. No abstract available. PMID: 15180242 [PubMed - indexed for MEDLINE]Related articlesFree article

On the molecular pathology of neurodegeneration in IMPDH1-based retinitis pigmentosa.

Aherne A, Kennan A, Kenna PF, McNally N, Lloyd DG, Alberts IL, Kiang AS, Humphries MM, Ayuso C, Engel PC, Gu JJ, Mitchell BS, Farrar GJ, Humphries P.

Hum Mol Genet. 2004 Mar 15;13(6):641-50.PMID: 14981049 [PubMed - indexed for MEDLINE]Related articlesFree article

Transplantation of syngeneic Schwann cells to the retina of the rhodopsin knockout (rho(-/-)) mouse.

Keegan DJ, Kenna P, Humphries MM, Humphries P, Flitcroft DI, Coffey PJ, Lund RD, Lawrence JM.

Invest Ophthalmol Vis Sci. 2003 Aug;44(8):3526-32.PMID: 12882803 [PubMed - indexed for MEDLINE]Related articlesFree article

Sensitivity of photoreceptor-derived cell line (661W) to baculoviral p35, Z-VAD.FMK, and Fas-associated death domain.

Tuohy G, Millington-Ward S, Kenna PF, Humphries P, Farrar GJ.

Invest Ophthalmol Vis Sci. 2002 Nov;43(11):3583-9.PMID: 12407171 [PubMed - indexed for MEDLINE]Related articlesFree article

Validation in mesenchymal progenitor cells of a mutation-independent ex vivo approach to gene therapy for osteogenesis imperfecta.

Millington-Ward S, Allers C, Tuohy G, Conget P, Allen D, McMahon HP, Kenna PF, Humphries P, Farrar GJ.

Hum Mol Genet. 2002 Sep 15;11(19):2201-6.PMID: 12217948 [PubMed - indexed for MEDLINE]Related articlesFree article

Murine model of autosomal dominant retinitis pigmentosa generated by targeted deletion at codon 307 of the rds-peripherin gene.

McNally N, Kenna PF, Rancourt D, Ahmed T, Stitt A, Colledge WH, Lloyd DG, Palfi A, O'Neill B, Humphries MM, Humphries P, Farrar GJ.

Hum Mol Genet. 2002 May 1;11(9):1005-16.PMID: 11978760 [PubMed - indexed for MEDLINE]Related articlesFree article

Identification of an IMPDH1 mutation in autosomal dominant retinitis pigmentosa (RP10) revealed following comparative microarray analysis of transcripts derived from retinas of wild-type and Rho(-/-) mice.

Kennan A, Aherne A, Palfi A, Humphries M, McKee A, Stitt A, Simpson DA, Demtroder K, Orntoft T, Ayuso C, Kenna PF, Farrar GJ, Humphries P.

Hum Mol Genet. 2002 Mar 1;11(5):547-57.PMID: 11875049 [PubMed - indexed for MEDLINE]Related articlesFree article

On the genetics of retinitis pigmentosa and on mutation-independent approaches to therapeutic intervention.

Farrar GJ, Kenna PF, Humphries P.

EMBO J. 2002 Mar 1;21(5):857-64. Review.PMID: 11867514 [PubMed - indexed for MEDLINE]Related articlesFree article

Comparative structural and functional analysis of photoreceptor neurons of Rho-/- mice reveal increased survival on C57BL/6J in comparison to 129Sv genetic background.

Humphries MM, Kiang S, McNally N, Donovan MA, Sieving PA, Bush RA, Machida S, Cotter T, Hobson A, Farrar J, Humphries P, Kenna P.

Vis Neurosci. 2001 May-Jun;18(3):437-43.PMID: 11497420 [PubMed - indexed for MEDLINE]Related articles

Apoptotic photoreceptor death in the rhodopsin knockout mouse in the presence and absence of c-fos.

Hobson AH, Donovan M, Humphries MM, Tuohy G, McNally N, Carmody R, Cotter T, Farrar GJ, Kenna PF, Humphries P.

Exp Eye Res. 2000 Sep;71(3):247-54.PMID: 10973734 [PubMed - indexed for MEDLINE]Related articles

Ribozyme-based therapeutic approaches for autosomal dominant retinitis pigmentosa.

O'Neill B, Millington-Ward S, O'Reilly M, Tuohy G, Kiang AS, Kenna PF, Humphries P, Farrar GJ.

Invest Ophthalmol Vis Sci. 2000 Sep;41(10):2863-9.PMID: 10967039 [PubMed - indexed for MEDLINE]Related articlesFree article

A mutation-independent therapeutic strategem for osteogenesis imperfecta.

Millington-Ward S, O'Neill B, Kiang AS, Humphries P, Kenna PF, Farrar GJ.

Antisense Nucleic Acid Drug Dev. 1999 Dec;9(6):537-42.PMID: 10645780 [PubMed - indexed for MEDLINE]Related articles

Structural and functional rescue of murine rod photoreceptors by human rhodopsin transgene.

McNally N, Kenna P, Humphries MM, Hobson AH, Khan NW, Bush RA, Sieving PA, Humphries P, Farrar GJ.

Hum Mol Genet. 1999 Jul;8(7):1309-12.PMID: 10369877 [PubMed - indexed for MEDLINE]Related articlesFree article

Retinitis pigmentosa and progressive sensorineural hearing loss caused by a C12258A mutation in the mitochondrial MTTS2 gene.

Mansergh FC, Millington-Ward S, Kennan A, Kiang AS, Humphries M, Farrar GJ, Humphries P, Kenna PF.

Am J Hum Genet. 1999 Apr;64(4):971-85.PMID: 10090882 [PubMed - indexed for MEDLINE]Related articlesFree article

A novel mutation within the rhodopsin gene (Thr-94-Ile) causing autosomal dominant congenital stationary night blindness.

al-Jandal N, Farrar GJ, Kiang AS, Humphries MM, Bannon N, Findlay JB, Humphries P, Kenna PF.

Hum Mutat. 1999;13(1):75-81.PMID: 9888392 [PubMed - indexed for MEDLINE]Related articles

Retinitis pigmentosa and schizophrenia.

McDonald C, Kenna P, Larkin T.

Eur Psychiatry. 1998 Dec;13(8):423-6.PMID: 19698660 [PubMed - in process]Related articles

A novel Asp380Ala mutation in the GLC1A/myocilin gene in a family with juvenile onset primary open angle glaucoma.

Kennan AM, Mansergh FC, Fingert JH, Clark T, Ayuso C, Kenna PF, Humphries P, Farrar GJ.

J Med Genet. 1998 Nov;35(11):957-60.PMID: 9832047 [PubMed - indexed for MEDLINE]Related articlesFree article

Giant retinal tear in Usher syndrome type II: coincidence or association?

Cahill MT, Barry PJ, Kenna PF.

Retina. 1998;18(2):177-8. No abstract available. PMID: 9564703 [PubMed - indexed for MEDLINE]Related articles

Novel mutations in the TIGR gene in early and late onset open angle glaucoma.

Mansergh FC, Kenna PF, Ayuso C, Kiang AS, Humphries P, Farrar GJ.

Hum Mutat. 1998;11(3):244-51.PMID: 9521427 [PubMed - indexed for MEDLINE]Related articles

Strategems in vitro for gene therapies directed to dominant mutations.

Millington-Ward S, O'Neill B, Tuohy G, Al-Jandal N, Kiang AS, Kenna PF, Palfi A, Hayden P, Mansergh F, Kennan A, Humphries P, Farrar GJ.

Hum Mol Genet. 1997 Sep;6(9):1415-26.PMID: 9285777 [PubMed - indexed for MEDLINE]Related articlesFree article

Clinical and molecular genetic characterisation of a family segregating autosomal dominant retinitis pigmentosa and sensorineural deafness.

Kenna P, Mansergh F, Millington-Ward S, Erven A, Kumar-Singh R, Brennan R, Farrar GJ, Humphries P.

Br J Ophthalmol. 1997 Mar;81(3):207-13.PMID: 9135384 [PubMed - indexed for MEDLINE]Related articlesFree article

Photoastigmatic refractive keratectomy for compound myopic astigmatism with a Nidek laser.

Goggin MJ, Kenna PF, Lavery FL.

J Refract Surg. 1997 Mar-Apr;13(2):162-6.PMID: 9109073 [PubMed - indexed for MEDLINE]Related articles

Retinopathy induced in mice by targeted disruption of the rhodopsin gene.

Humphries MM, Rancourt D, Farrar GJ, Kenna P, Hazel M, Bush RA, Sieving PA, Sheils DM, McNally N, Creighton P, Erven A, Boros A, Gulya K, Capecchi MR, Humphries P.

Nat Genet. 1997 Feb;15(2):216-9.PMID: 9020854 [PubMed - indexed for MEDLINE]Related articles

Haze following photorefractive and photoastigmatic refractive keratectomy with the Nidek EC5000 and the Summit ExciMed UV200.

Goggin M, Kenna P, Lavery F.

J Cataract Refract Surg. 1997 Jan-Feb;23(1):50-3.PMID: 9100107 [PubMed - indexed for MEDLINE]Related articles

Three keratin gene mutations account for the majority of dominant simplex epidermolysis bullosa cases within the population of Ireland.

Humphries MM, Mansergh FC, Kiang AS, Jordan SA, Sheils DM, Martin MJ, Farrar GJ, Kenna PF, Young MM, Humphries P.

Hum Mutat. 1996;8(1):57-63.PMID: 8807337 [PubMed - indexed for MEDLINE]Related articles

Evidence for genetic heterogeneity in Best's vitelliform macular dystrophy.

Mansergh FC, Kenna PF, Rudolph G, Meitinger T, Farrar GJ, Kumar-Singh R, Scorer J, Hally AM, Mynett-Johnson L, Humphries MM.

J Med Genet. 1995 Nov;32(11):855-8.PMID: 8592326 [PubMed - indexed for MEDLINE]Related articlesFree article

New dimensions in macular dystrophies.

Humphries P, Kenna P, Farrar GJ.

Nat Genet. 1994 Dec;8(4):315-7. No abstract available. PMID: 7894477 [PubMed - indexed for MEDLINE]Related articles

Exclusion of the involvement of all known retinitis pigmentosa loci in the disease present in a family of Irish origin provides evidence for a sixth autosomal dominant locus (RP8).

Kumar-Singh R, Farrar GJ, Mansergh F, Kenna P, Bhattacharya S, Gal A, Humphries P.

Hum Mol Genet. 1993 Jul;2(7):875-8.PMID: 8364569 [PubMed - indexed for MEDLINE]Related articles

Localization of an autosomal dominant retinitis pigmentosa gene to chromosome 7q.

Jordan SA, Farrar GJ, Kenna P, Humphries MM, Sheils DM, Kumar-Singh R, Sharp EM, Soriano N, Ayuso C, Benitez J, et al.

Nat Genet. 1993 May;4(1):54-8.PMID: 8513324 [PubMed - indexed for MEDLINE]Related articles