Utrophin up-regulation by an artificial transcription factor in transgenic mice (Articolo in rivista)

Type

• Articolo in rivista (Classe)
• Prodotto della ricerca (Classe)

Label

• Utrophin up-regulation by an artificial transcription factor in transgenic mice (Articolo in rivista) (literal)

Anno

• 2007-01-01T00:00:00+01:00 (literal)

Alternative label

• Mattei E; Corbi N; Di Certo MG; Strimpakos G; Severini C; Onori A; Desantis A; Libri V; Buontempo S; Floridi A; Fanciulli M; Baban D; Davies KE; Passananti C. (2007)
  Utrophin up-regulation by an artificial transcription factor in transgenic mice
  in PloS one
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Mattei E; Corbi N; Di Certo MG; Strimpakos G; Severini C; Onori A; Desantis A; Libri V; Buontempo S; Floridi A; Fanciulli M; Baban D; Davies KE; Passananti C. (literal)

Pagina inizio

• e774 (literal)

Pagina fine

• - (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 2 (literal)

Rivista

• PloS one (Rivista)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#note

• Published online 2007 August 22 (literal)

Note

• ISI Web of Science (WOS) (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• 1 = Istituto di Neurobiologia e Medicina Molecolare, Consiglio Nazionale delle Ricerche, European Brain Research Institute, Rome, Italy; 2 = Italian Association for Cancer Research, Roman Oncogenomic Center, Rome, Italy; 3 = Istituto di Biologia e Patologia Molecolari, Consiglio Nazionale delle Ricerche, Regina Elena Cancer Institute, Rome, Italy; 4 = Department of Experimental Medicine, University of L'Aquila, L'Aquila, Italy; 5 = Laboratory B, Regina Elena Cancer Institute, Rome, Italy; 6 = Department of Physiology, Anatomy and Genetics, Medical Research Council Functional Genetics Unit, University of Oxford, Oxford, United Kingdom. (literal)

Titolo

• Utrophin up-regulation by an artificial transcription factor in transgenic mice (literal)

Abstract

• Duchenne Muscular Dystrophy (DMD) is a severe muscle degenerative disease, due to absence of dystrophin. There is currently no effective treatment for DMD. Our aim is to up-regulate the expression level of the dystrophin related gene utrophin in DMD, complementing in this way the lack of dystrophin functions. To this end we designed and engineered several synthetic zinc finger based transcription factors. In particular, we have previously shown that the artificial three zinc finger protein named Jazz, fused with the appropriate effector domain, is able to drive the transcription of a test gene from the utrophin promoter “A”. Here we report on the characterization of Vp16-Jazz-transgenic mice that specifically over-express the utrophin gene at the muscular level. A Chromatin Immunoprecipitation assay (ChIP) demonstrated the effective access/binding of the Jazz protein to active chromatin in mouse muscle and Vp16-Jazz was shown to be able to up-regulate endogenous utrophin gene expression by immunohistochemistry, western blot analyses and real-time PCR. To our knowledge, this is the first example of a transgenic mouse expressing an artificial gene coding for a zinc finger based transcription factor. The achievement of Vp16-Jazz transgenic mice validates the strategy of transcriptional targeting of endogenous genes and could represent an exclusive animal model for use in drug discovery and therapeutics. (literal)

Prodotto di

• Istituto di neurobiologia e medicina molecolare (INMM) (Istituto)
• Patologie del S.N. e Fattori di Crescita (ME.P02.010.001) (Modulo)
• Institute of molecular biology and pathology (IBPM) (Istituto)
• Controllo di proliferazione, differenziamento e morte cellulare (SV.P15.010.001) (Modulo)

Autore CNR

• CLAUDIO PASSANANTI (Unità di personale interno)
• ANNALISA ONORI (Persona)
• MARIA GRAZIA DI CERTO (Persona)
• NICOLETTA CORBI (Persona)
• ELISABETTA MATTEI (Persona)
• CINZIA SEVERINI (Persona)

Incoming links:

Prodotto

• Institute of molecular biology and pathology (IBPM) (Istituto)
• Istituto di neurobiologia e medicina molecolare (INMM) (Istituto)
• Controllo di proliferazione, differenziamento e morte cellulare (SV.P15.010.001) (Modulo)
• Patologie del S.N. e Fattori di Crescita (ME.P02.010.001) (Modulo)

Autore CNR di

• CINZIA SEVERINI (Persona)
• CLAUDIO PASSANANTI (Unità di personale interno)
• ELISABETTA MATTEI (Persona)
• ANNALISA ONORI (Persona)
• MARIA GRAZIA DI CERTO (Persona)
• NICOLETTA CORBI (Persona)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• PloS one (Rivista)