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Items: 1 to 50 of 63

1.

Homozygous Recessive Versican Missense Variation Is Associated With Early Teeth Loss in a Pakistani Family.

Bigoni S, Neri M, Scotton C, Farina R, Sabatelli P, Jiang C, Zhang J, Falzarano MS, Rossi R, Ognibene D, Selvatici R, Gualandi F, Bosshardt D, Perri P, Campa C, Brancati F, Salvatore M, De Stefano MC, Taruscio D, Trombelli L, Fang M, Ferlini A.

Front Genet. 2019 Jan 21;9:723. doi: 10.3389/fgene.2018.00723. eCollection 2018.

2.

Improved RE31 Analogues Containing Modified Nucleic Acid Monomers: Thermodynamic, Structural, and Biological Effects.

Kotkowiak W, Wengel J, Scotton CJ, Pasternak A.

J Med Chem. 2019 Mar 14;62(5):2499-2507. doi: 10.1021/acs.jmedchem.8b01806. Epub 2019 Feb 22.

PMID:
30735377
3.

Biocompatibility and toxicity of graphene quantum dots for potential application in photodynamic therapy.

Tabish TA, Scotton CJ, Ferguson DCJ, Lin L, der Veen AV, Lowry S, Ali M, Jabeen F, Ali M, Winyard PG, Zhang S.

Nanomedicine (Lond). 2018 Aug 1;13(15):1923-1937. doi: 10.2217/nnm-2018-0018. Epub 2018 Aug 20.

4.

Transcriptional and epigenetic analyses of the DMD locus reveal novel cis‑acting DNA elements that govern muscle dystrophin expression.

Gherardi S, Bovolenta M, Passarelli C, Falzarano MS, Pigini P, Scotton C, Neri M, Armaroli A, Osman H, Selvatici R, Gualandi F, Recchia A, Mora M, Bernasconi P, Maggi L, Morandi L, Ferlini A, Perini G.

Biochim Biophys Acta Gene Regul Mech. 2017 Nov;1860(11):1138-1147. doi: 10.1016/j.bbagrm.2017.08.010. Epub 2017 Sep 1.

PMID:
28867298
5.

Molecular epidemiology of Pseudomonas aeruginosa in an unsegregated bronchiectasis cohort sharing hospital facilities with a cystic fibrosis cohort.

Mitchelmore PJ, Randall J, Bull MJ, Moore KA, O'Neill PA, Paszkiewicz K, Mahenthiralingam E, Scotton CJ, Sheldon CD, Withers NJ, Brown AR.

Thorax. 2017 Aug 26. pii: thoraxjnl-2016-209889. doi: 10.1136/thoraxjnl-2016-209889. [Epub ahead of print]

PMID:
28844058
6.

Delving Deep into the Proteome of Lung Fibrosis Brings Plasma Cells to the Surface.

Gibbons MA, Scotton CJ.

Am J Respir Crit Care Med. 2017 Nov 15;196(10):1238-1240. doi: 10.1164/rccm.201706-1186ED. No abstract available.

PMID:
28696776
7.

Differential Expression of VEGF-Axxx Isoforms Is Critical for Development of Pulmonary Fibrosis.

Barratt SL, Blythe T, Jarrett C, Ourradi K, Shelley-Fraser G, Day MJ, Qiu Y, Harper S, Maher TM, Oltean S, Hames TJ, Scotton CJ, Welsh GI, Bates DO, Millar AB.

Am J Respir Crit Care Med. 2017 Aug 15;196(4):479-493. doi: 10.1164/rccm.201603-0568OC.

8.

Complex phenotypes associated with STIM1 mutations in both coiled coil and EF-hand domains.

Harris E, Burki U, Marini-Bettolo C, Neri M, Scotton C, Hudson J, Bertoli M, Evangelista T, Vroling B, Polvikoski T, Roberts M, Töpf A, Bushby K, McArthur D, Lochmüller H, Ferlini A, Straub V, Barresi R.

Neuromuscul Disord. 2017 Sep;27(9):861-872. doi: 10.1016/j.nmd.2017.05.002. Epub 2017 May 4.

PMID:
28624464
9.

Recessive mutations in MSTO1 cause mitochondrial dynamics impairment, leading to myopathy and ataxia.

Nasca A, Scotton C, Zaharieva I, Neri M, Selvatici R, Magnusson OT, Gal A, Weaver D, Rossi R, Armaroli A, Pane M, Phadke R, Sarkozy A, Muntoni F, Hughes I, Cecconi A, Hajnóczky G, Donati A, Mercuri E, Zeviani M, Ferlini A, Ghezzi D.

Hum Mutat. 2017 Aug;38(8):970-977. doi: 10.1002/humu.23262. Epub 2017 Jun 6.

10.

TGFβ upregulates PAR-1 expression and signalling responses in A549 lung adenocarcinoma cells.

Smoktunowicz N, Platé M, Stern AO, D'Antongiovanni V, Robinson E, Chudasama V, Caddick S, Scotton CJ, Jarai G, Chambers RC.

Oncotarget. 2016 Oct 4;7(40):65471-65484. doi: 10.18632/oncotarget.11472.

11.

Duchenne Muscular Dystrophy Myogenic Cells from Urine-Derived Stem Cells Recapitulate the Dystrophin Genotype and Phenotype.

Falzarano MS, D'Amario D, Siracusano A, Massetti M, Amodeo A, La Neve F, Maroni CR, Mercuri E, Osman H, Scotton C, Armaroli A, Rossi R, Selvatici R, Crea F, Ferlini A.

Hum Gene Ther. 2016 Oct;27(10):772-783. doi: 10.1089/hum.2016.079.

PMID:
27530229
12.

Developing a Collaborative Agenda for Humanities and Social Scientific Research on Laboratory Animal Science and Welfare.

Davies GF, Greenhough BJ, Hobson-West P, Kirk RG, Applebee K, Bellingan LC, Berdoy M, Buller H, Cassaday HJ, Davies K, Diefenbacher D, Druglitrø T, Escobar MP, Friese C, Herrmann K, Hinterberger A, Jarrett WJ, Jayne K, Johnson AM, Johnson ER, Konold T, Leach MC, Leonelli S, Lewis DI, Lilley EJ, Longridge ER, McLeod CM, Miele M, Nelson NC, Ormandy EH, Pallett H, Poort L, Pound P, Ramsden E, Roe E, Scalway H, Schrader A, Scotton CJ, Scudamore CL, Smith JA, Whitfield L, Wolfensohn S.

PLoS One. 2016 Jul 18;11(7):e0158791. doi: 10.1371/journal.pone.0158791. eCollection 2016.

13.

The Popeye Domain Containing Genes and their Function in Striated Muscle.

Schindler RF, Scotton C, French V, Ferlini A, Brand T.

J Cardiovasc Dev Dis. 2016 Jun 15;3(2):22.

14.

The Popeye Domain Containing Genes and Their Function in Striated Muscle.

Schindler RFR, Scotton C, French V, Ferlini A, Brand T.

J Cardiovasc Dev Dis. 2016 Jun 15;3(2). pii: E22. doi: 10.3390/jcdd3020022. Review.

15.

Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathy.

Scotton C, Bovolenta M, Schwartz E, Falzarano MS, Martoni E, Passarelli C, Armaroli A, Osman H, Rodolico C, Messina S, Pegoraro E, D'Amico A, Bertini E, Gualandi F, Neri M, Selvatici R, Boffi P, Maioli MA, Lochmüller H, Straub V, Bushby K, Castrignanò T, Pesole G, Sabatelli P, Merlini L, Braghetta P, Bonaldo P, Bernardi P, Foley R, Cirak S, Zaharieva I, Muntoni F, Capitanio D, Gelfi C, Kotelnikova E, Yuryev A, Lebowitz M, Zhang X, Hodge BA, Esser KA, Ferlini A.

J Cell Sci. 2016 Apr 15;129(8):1671-84. doi: 10.1242/jcs.175927. Epub 2016 Mar 4.

16.

POPDC1(S201F) causes muscular dystrophy and arrhythmia by affecting protein trafficking.

Schindler RF, Scotton C, Zhang J, Passarelli C, Ortiz-Bonnin B, Simrick S, Schwerte T, Poon KL, Fang M, Rinné S, Froese A, Nikolaev VO, Grunert C, Müller T, Tasca G, Sarathchandra P, Drago F, Dallapiccola B, Rapezzi C, Arbustini E, Di Raimo FR, Neri M, Selvatici R, Gualandi F, Fattori F, Pietrangelo A, Li W, Jiang H, Xu X, Bertini E, Decher N, Wang J, Brand T, Ferlini A.

J Clin Invest. 2016 Jan;126(1):239-53. doi: 10.1172/JCI79562. Epub 2015 Dec 7.

17.

Platform Technologies for Directly Reconstructing 3D Living Biomaterials.

Jayasinghe SN, Auguste J, Scotton CJ.

Adv Mater. 2015 Dec 16;27(47):7794-9. doi: 10.1002/adma.201503001. Epub 2015 Oct 28.

PMID:
26508202
18.

Duchenne Muscular Dystrophy: From Diagnosis to Therapy.

Falzarano MS, Scotton C, Passarelli C, Ferlini A.

Molecules. 2015 Oct 7;20(10):18168-84. doi: 10.3390/molecules201018168. Review.

19.

The anti-fibrotic effect of inhibition of TGFβ-ALK5 signalling in experimental pulmonary fibrosis in mice is attenuated in the presence of concurrent γ-herpesvirus infection.

Smoktunowicz N, Alexander RE, Franklin L, Williams AE, Holman B, Mercer PF, Jarai G, Scotton CJ, Chambers RC.

Dis Model Mech. 2015 Sep;8(9):1129-39. doi: 10.1242/dmm.019984. Epub 2015 Jul 2.

20.

Paternal germline mosaicism in collagen VI related myopathies.

Armaroli A, Trabanelli C, Scotton C, Venturoli A, Selvatici R, Brisca G, Merlini L, Bruno C, Ferlini A, Gualandi F.

Eur J Paediatr Neurol. 2015 Sep;19(5):533-6. doi: 10.1016/j.ejpn.2015.04.002. Epub 2015 Apr 30.

PMID:
25978941
21.

Validation of genetic modifiers for Duchenne muscular dystrophy: a multicentre study assessing SPP1 and LTBP4 variants.

van den Bergen JC, Hiller M, Böhringer S, Vijfhuizen L, Ginjaar HB, Chaouch A, Bushby K, Straub V, Scoto M, Cirak S, Humbertclaude V, Claustres M, Scotton C, Passarelli C, Lochmüller H, Muntoni F, Tuffery-Giraud S, Ferlini A, Aartsma-Rus AM, Verschuuren JJ, 't Hoen PA, Spitali P.

J Neurol Neurosurg Psychiatry. 2015 Oct;86(10):1060-5. doi: 10.1136/jnnp-2014-308409. Epub 2014 Dec 4.

22.

Muscle proteomics reveals novel insights into the pathophysiological mechanisms of collagen VI myopathies.

De Palma S, Capitanio D, Vasso M, Braghetta P, Scotton C, Bonaldo P, Lochmüller H, Muntoni F, Ferlini A, Gelfi C.

J Proteome Res. 2014 Nov 7;13(11):5022-30. doi: 10.1021/pr500675e. Epub 2014 Sep 24.

PMID:
25211533
23.

Intrinsic defence capacity and therapeutic potential of natriuretic peptides in pulmonary hypertension associated with lung fibrosis.

Baliga RS, Scotton CJ, Trinder SL, Chambers RC, MacAllister RJ, Hobbs AJ.

Br J Pharmacol. 2014 Jul;171(14):3463-75. doi: 10.1111/bph.12694.

24.

Biomarkers in rare diseases.

Ferlini A, Scotton C, Novelli G.

Public Health Genomics. 2013;16(6):313-21. doi: 10.1159/000355938. Epub 2014 Feb 3. Review.

PMID:
24503592
25.

Proteinase activated receptor 1 mediated fibrosis in a mouse model of liver injury: a role for bone marrow derived macrophages.

Kallis YN, Scotton CJ, Mackinnon AC, Goldin RD, Wright NA, Iredale JP, Chambers RC, Forbes SJ.

PLoS One. 2014 Jan 27;9(1):e86241. doi: 10.1371/journal.pone.0086241. eCollection 2014.

26.

Biomarkers in rare neuromuscular diseases.

Scotton C, Passarelli C, Neri M, Ferlini A.

Exp Cell Res. 2014 Jul 1;325(1):44-9. doi: 10.1016/j.yexcr.2013.12.020. Epub 2013 Dec 31. Review.

PMID:
24389168
27.

Evidence for a functional thymic stromal lymphopoietin signaling axis in fibrotic lung disease.

Datta A, Alexander R, Sulikowski MG, Nicholson AG, Maher TM, Scotton CJ, Chambers RC.

J Immunol. 2013 Nov 1;191(9):4867-79. doi: 10.4049/jimmunol.1300588. Epub 2013 Sep 30.

28.

Exome sequencing in a family with intellectual disability, early onset spasticity, and cerebellar atrophy detects a novel mutation in EXOSC3.

Zanni G, Scotton C, Passarelli C, Fang M, Barresi S, Dallapiccola B, Wu B, Gualandi F, Ferlini A, Bertini E, Wei W.

Neurogenetics. 2013 Nov;14(3-4):247-50. doi: 10.1007/s10048-013-0371-z. Epub 2013 Aug 24.

PMID:
23975261
29.

Proteinase-activated receptor-1, CCL2, and CCL7 regulate acute neutrophilic lung inflammation.

Mercer PF, Williams AE, Scotton CJ, José RJ, Sulikowski M, Moffatt JD, Murray LA, Chambers RC.

Am J Respir Cell Mol Biol. 2014 Jan;50(1):144-57. doi: 10.1165/rcmb.2013-0142OC.

30.

SERCA1 protein expression in muscle of patients with Brody disease and Brody syndrome and in cultured human muscle fibers.

Guglielmi V, Vattemi G, Gualandi F, Voermans NC, Marini M, Scotton C, Pegoraro E, Oosterhof A, Kósa M, Zádor E, Valente EM, De Grandis D, Neri M, Codemo V, Novelli A, van Kuppevelt TH, Dallapiccola B, van Engelen BG, Ferlini A, Tomelleri G.

Mol Genet Metab. 2013 Sep-Oct;110(1-2):162-9. doi: 10.1016/j.ymgme.2013.07.015. Epub 2013 Jul 20.

PMID:
23911890
31.

Ratings can improve standards.

Scotton C.

Nurs Older People. 2013 Mar;25(2):8. No abstract available.

PMID:
23581216
32.

A patient with limb girdle muscular dystrophy carries a TRIM32 deletion, detected by a novel CGH array, in compound heterozygosis with a nonsense mutation.

Neri M, Selvatici R, Scotton C, Trabanelli C, Armaroli A, De Grandis D, Levy N, Gualandi F, Ferlini A.

Neuromuscul Disord. 2013 Jun;23(6):478-82. doi: 10.1016/j.nmd.2013.02.003. Epub 2013 Mar 28.

PMID:
23541687
33.

Ex vivo micro-computed tomography analysis of bleomycin-induced lung fibrosis for preclinical drug evaluation.

Scotton CJ, Hayes B, Alexander R, Datta A, Forty EJ, Mercer PF, Blanchard A, Chambers RC.

Eur Respir J. 2013 Dec;42(6):1633-45. doi: 10.1183/09031936.00182412. Epub 2013 Mar 21.

34.

The DMD locus harbours multiple long non-coding RNAs which orchestrate and control transcription of muscle dystrophin mRNA isoforms.

Bovolenta M, Erriquez D, Valli E, Brioschi S, Scotton C, Neri M, Falzarano MS, Gherardi S, Fabris M, Rimessi P, Gualandi F, Perini G, Ferlini A.

PLoS One. 2012;7(9):e45328. doi: 10.1371/journal.pone.0045328. Epub 2012 Sep 21.

35.

Genetic characterization in symptomatic female DMD carriers: lack of relationship between X-inactivation, transcriptional DMD allele balancing and phenotype.

Brioschi S, Gualandi F, Scotton C, Armaroli A, Bovolenta M, Falzarano MS, Sabatelli P, Selvatici R, D'Amico A, Pane M, Ricci G, Siciliano G, Tedeschi S, Pini A, Vercelli L, De Grandis D, Mercuri E, Bertini E, Merlini L, Mongini T, Ferlini A.

BMC Med Genet. 2012 Aug 16;13:73.

36.

Coagulation cascade proteinases in lung injury and fibrosis.

Chambers RC, Scotton CJ.

Proc Am Thorac Soc. 2012 Jul;9(3):96-101. doi: 10.1513/pats.201201-006AW. Review.

PMID:
22802281
37.

Brody syndrome: a clinically heterogeneous entity distinct from Brody disease: a review of literature and a cross-sectional clinical study in 17 patients.

Voermans NC, Laan AE, Oosterhof A, van Kuppevelt TH, Drost G, Lammens M, Kamsteeg EJ, Scotton C, Gualandi F, Guglielmi V, van den Heuvel L, Vattemi G, van Engelen BG.

Neuromuscul Disord. 2012 Nov;22(11):944-54. doi: 10.1016/j.nmd.2012.03.012. Epub 2012 Jun 15.

PMID:
22704959
38.

Innate immunity in lung fibrosis: a therapeutic role for surfactant protein D?

Scotton CJ, Chambers RC.

Am J Respir Crit Care Med. 2012 Mar 1;185(5):471-3. doi: 10.1164/rccm.201112-2193ED. No abstract available.

PMID:
22383565
39.

Rapid, comprehensive analysis of the dystrophin transcript by a custom micro-fluidic exome array.

Bovolenta M, Scotton C, Falzarano MS, Gualandi F, Ferlini A.

Hum Mutat. 2012 Mar;33(3):572-81. doi: 10.1002/humu.22017. Epub 2012 Jan 25.

PMID:
22223181
40.

Progressive severe lung injury by zinc oxide nanoparticles; the role of Zn2+ dissolution inside lysosomes.

Cho WS, Duffin R, Howie SE, Scotton CJ, Wallace WA, Macnee W, Bradley M, Megson IL, Donaldson K.

Part Fibre Toxicol. 2011 Sep 6;8:27. doi: 10.1186/1743-8977-8-27.

41.

Bio-electrosprayed living composite matrix implanted into mouse models.

Jayasinghe SN, Warnes G, Scotton CJ.

Macromol Biosci. 2011 Oct 10;11(10):1364-9. doi: 10.1002/mabi.201100131. Epub 2011 Jul 13.

PMID:
21755598
42.

In search of the fibrotic epithelial cell: opportunities for a collaborative network.

Jenkins G, Blanchard A, Borok Z, Bradding P, Ehrhardt C, Fisher A, Hirani N, Johnson S, Königshoff M, Maher TM, Millar A, Parfrey H, Scotton C, Tetley T, Thickett D, Wolters P; ECIPF workshop.

Thorax. 2012 Feb;67(2):179-82. doi: 10.1136/thoraxjnl-2011-200195. Epub 2011 Jun 15.

PMID:
21680569
43.

Novel therapeutic approaches for pulmonary fibrosis.

Datta A, Scotton CJ, Chambers RC.

Br J Pharmacol. 2011 May;163(1):141-72. doi: 10.1111/j.1476-5381.2011.01247.x. Review.

44.

Bleomycin revisited: towards a more representative model of IPF?

Scotton CJ, Chambers RC.

Am J Physiol Lung Cell Mol Physiol. 2010 Oct;299(4):L439-41. doi: 10.1152/ajplung.00258.2010. Epub 2010 Jul 30. No abstract available.

45.

Bone marrow stem cells expressing keratinocyte growth factor via an inducible lentivirus protects against bleomycin-induced pulmonary fibrosis.

Aguilar S, Scotton CJ, McNulty K, Nye E, Stamp G, Laurent G, Bonnet D, Janes SM.

PLoS One. 2009 Nov 24;4(11):e8013. doi: 10.1371/journal.pone.0008013.

46.

Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury.

Scotton CJ, Krupiczojc MA, Königshoff M, Mercer PF, Lee YC, Kaminski N, Morser J, Post JM, Maher TM, Nicholson AG, Moffatt JD, Laurent GJ, Derian CK, Eickelberg O, Chambers RC.

J Clin Invest. 2009 Sep;119(9):2550-63. doi: 10.1172/JCI33288. Epub 2009 Aug 3.

47.

Absence of proteinase-activated receptor-1 signaling in mice confers protection from fMLP-induced goblet cell metaplasia.

Atzori L, Lucattelli M, Scotton CJ, Laurent GJ, Bartalesi B, De Cunto G, Lunghi B, Chambers RC, Lungarella G.

Am J Respir Cell Mol Biol. 2009 Dec;41(6):680-7. doi: 10.1165/rcmb.2007-0386OC. Epub 2009 Mar 23.

48.

Pulmonary epithelium is a prominent source of proteinase-activated receptor-1-inducible CCL2 in pulmonary fibrosis.

Mercer PF, Johns RH, Scotton CJ, Krupiczojc MA, Königshoff M, Howell DC, McAnulty RJ, Das A, Thorley AJ, Tetley TD, Eickelberg O, Chambers RC.

Am J Respir Crit Care Med. 2009 Mar 1;179(5):414-25. doi: 10.1164/rccm.200712-1827OC. Epub 2008 Dec 5.

49.

Modeling the impact of HIV chemoprophylaxis strategies among men who have sex with men in the United States: HIV infections prevented and cost-effectiveness.

Desai K, Sansom SL, Ackers ML, Stewart SR, Hall HI, Hu DJ, Sanders R, Scotton CR, Soorapanth S, Boily MC, Garnett GP, McElroy PD.

AIDS. 2008 Sep 12;22(14):1829-39. doi: 10.1097/QAD.0b013e32830e00f5.

PMID:
18753932
50.

Coagulation signalling following tissue injury: focus on the role of factor Xa.

Krupiczojc MA, Scotton CJ, Chambers RC.

Int J Biochem Cell Biol. 2008;40(6-7):1228-37. doi: 10.1016/j.biocel.2008.02.026. Epub 2008 Mar 7. Review.

PMID:
18420447

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