Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer.

Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer.

Utrophin is highly homologous and structurally similar to dystrophin, and in gene delivery experiments in mdx mice was able to functionally replace dystrophin. We performed mini-utrophin gene transfer in Golden Retriever dogs with canine muscular dystrophy (CXMD). Unlike the mouse model, the clinico...

Fuld beskrivelse

Bibliografiske detaljer
Main Authors:	Cerletti, M, Negri, T, Cozzi, F, Colpo, R, Andreetta, F, Croci, D, Davies, K, Cornelio, F, Pozza, O, Karpati, G, Gilbert, R, Mora, M
Format:	Journal article
Sprog:	English
Udgivet:	2003

Lignende værker

Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene.
af: Wakefield, P, et al.
Udgivet: (2000)

Utrophin upregulation in Duchenne muscular dystrophy.
af: Hirst, R, et al.
Udgivet: (2005)

Utrophin in the therapy of Duchenne Muscular Dystrophy
af: Potter, A, et al.
Udgivet: (2006)

The role of utrophin in the potential therapy of Duchenne muscular dystrophy.
af: Perkins, K, et al.
Udgivet: (2002)

Discovery of 2-arylbenzoxazoles as upregulators of utrophin production for the treatment of Duchenne muscular dystrophy.
af: Chancellor, DR, et al.
Udgivet: (2011)

The potential of utrophin and dystrophin combination therapies for Duchenne muscular dystrophy
af: Guiraud, S, et al.
Udgivet: (2019)

Identification of new chemical compounds with upregulate utrophin for the therapy of Duchenne muscular dystrophy
af: Fairclough, R, et al.
Udgivet: (2012)

Mechanism of action studies of the utrophin modulator ezutromid for the treatment of Duchenne muscular dystrophy
af: Wilkinson, I
Udgivet: (2019)

Metabolic profiles of dystrophin and utrophin expression in mouse models of Duchenne muscular dystrophy.
af: Griffin, J, et al.
Udgivet: (2002)

Promising therapeutic approaches of utrophin replacing dystrophin in the treatment of Duchenne muscular dystrophy
af: Ruo Wu, et al.
Udgivet: (2022-11-01)

Evaluation of novel compounds for upregulation of utrophin in animal models of Duchenne muscular dystrophy therapy
af: Fairclough, R, et al.
Udgivet: (2008)

Utrophin upregulation and microRNAs - two avenues of Duchenne muscular dystrophy therapy research
af: Bareja, A
Udgivet: (2011)

Expression of different isoforms of utrophin in human muscle; potentials for therapy of Duchenne muscular dystrophy
af: Sewry, C, et al.
Udgivet: (2004)

Utrophin in therapy of Duchenne muscular distrophy
af: Fisher, R
Udgivet: (2001)

Utrophin influences mitochondrial pathology and oxidative stress in dystrophic muscle
af: Kennedy, T, et al.
Udgivet: (2017)

Utrophin influences mitochondrial pathology and oxidative stress in dystrophic muscle
af: Tahnee L. Kennedy, et al.
Udgivet: (2017-10-01)

New orally available compounds which modulate utrophin expression for the therapy of Duchenne muscular dystrophy (DMD)
af: Fairclough, R, et al.
Udgivet: (2014)

Correlation of utrophin levels with the dystrophin protein complex and muscle fibre regeneration in Duchenne and Becker muscular dystrophy muscle biopsies
af: Janghra, N, et al.
Udgivet: (2016)

Analysis of the transcriptional control of utrophin promoters as a therapeutic strategy for Duchenne muscular dystrophy (DMD).
af: Perkins, K, et al.
Udgivet: (2000)

Inactivation of Sirt6 ameliorates muscular dystrophy in mdx mice by releasing suppression of utrophin expression
af: Angelina M. Georgieva, et al.
Udgivet: (2022-07-01)

Up-regulation of utrophin alleviates the dystrophic phenotype in the mdx mouse heart
af: Sang, E, et al.
Udgivet: (2000)

Future clinical and biomarker development for SMTC1100, the first utrophin modulator to enter clinical trials for Duchenne Muscular Dystrophy (DMD)
af: Tinsley, J, et al.
Udgivet: (2013)

Functional improvement of dystrophic muscle by repression of utrophin: let-7c interaction.
af: Manoj K Mishra, et al.
Udgivet: (2017-01-01)

Alternative utrophin mRNAs contribute to phenotypic differences between dystrophin-deficient mice and Duchenne muscular dystrophy
af: Perkins, K, et al.
Udgivet: (2018)

Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice.
af: Amanda J Beck, et al.
Udgivet: (2011-01-01)

Pannexin 1 dysregulation in Duchenne muscular dystrophy and its exacerbation of dystrophic features in mdx mice
af: Emily Freeman, et al.
Udgivet: (2024-04-01)

Abnormal carbohydrate metabolism in a canine model for muscular dystrophy
af: Andressa R. Amaral, et al.
Udgivet: (2017-01-01)

Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat
af: Keitaro Yamanouchi, et al.
Udgivet: (2022-10-01)

Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies.
af: Narinder Janghra, et al.
Udgivet: (2016-01-01)

Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse.
af: Tinsley, J, et al.
Udgivet: (2011)

Glycine administration attenuates progression of dystrophic pathology in prednisolone-treated dystrophin/utrophin null mice
af: Daniel J. Ham, et al.
Udgivet: (2019-09-01)

Iron overload and impaired iron handling contribute to the dystrophic pathology in models of Duchenne muscular dystrophy
af: Francesca M. Alves, et al.
Udgivet: (2022-06-01)

The role of basal and myogenic factors in the transcriptional activation of utrophin promoter A: implications for therapeutic up-regulation in Duchenne muscular dystrophy.
af: Perkins, K, et al.
Udgivet: (2001)

Nitric Oxide and l-Arginine Cause an Accumulation of Utrophin at the Sarcolemma: A Possible Compensation for Dystrophin Loss in Duchenne Muscular Dystrophy
af: Emmanuel Chaubourt, et al.
Udgivet: (1999-12-01)

Prevention of dystrophic pathology in severely affected dystrophin/utrophin-deficient mice by morpholino-oligomer-mediated exon-skipping.
af: Goyenvalle, A, et al.
Udgivet: (2010)

Utrophin haploinsufficiency does not worsen the functional performance, resistance to eccentric contractions and force production of dystrophic mice.
af: Antoine Boulanger Piette, et al.
Udgivet: (2018-01-01)

Identification of therapeutics that target eEF1A2 and upregulate utrophin A translation in dystrophic muscles
af: Christine Péladeau, et al.
Udgivet: (2020-04-01)

Treatment with the anti-IL-6 receptor antibody attenuates muscular dystrophy via promoting skeletal muscle regeneration in dystrophin-/utrophin-deficient mice
af: Eiji Wada, et al.
Udgivet: (2017-10-01)

Chronic Dosing with Membrane Sealant Poloxamer 188 NF Improves Respiratory Dysfunction in Dystrophic Mdx and Mdx/Utrophin-/- Mice.
af: Bruce E Markham, et al.
Udgivet: (2015-01-01)

Bone measurements interact with phenotypic measures in canine Duchenne muscular dystrophy
af: Sarah M. Schneider, et al.
Udgivet: (2025-01-01)