The transgenic expression of LARGE exacerbates the muscle phenotype of dystroglycanopathy mice.
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Genetic Engineering of Dystroglycan in Animal Models of Muscular DystrophyAdeno-associated virus-mediated overexpression of LARGE rescues α-dystroglycan function in dystrophic mice with mutations in the fukutin-related protein.miRNA-based buffering of the cobblestone-lissencephaly-associated extracellular matrix receptor dystroglycan via its alternative 3'-UTR.Fukutin is prerequisite to ameliorate muscular dystrophic phenotype by myofiber-selective LARGE expression.How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouseTransgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?Postnatal Gene Therapy Improves Spatial Learning Despite the Presence of Neuronal Ectopia in a Model of Neuronal Migration Disorder.212th ENMC International Workshop: Animal models of congenital muscular dystrophies, Naarden, The Netherlands, 29-31 May 2015.What do mouse models of muscular dystrophy tell us about the DAPC and its components?Matriglycan: a novel polysaccharide that links dystroglycan to the basement membrane.Prenatal muscle development in a mouse model for the secondary dystroglycanopathiesAAV-mediated transfer of FKRP shows therapeutic efficacy in a murine model but requires control of gene expression.Overexpression of LARGE suppresses muscle regeneration via down-regulation of insulin-like growth factor 1 and aggravates muscular dystrophy in mice.Skeletal muscle contractile properties in a novel murine model for limb girdle muscular dystrophy 2i.Treating pediatric neuromuscular disorders: The future is now.Dose-Dependent Effects of FKRP Gene-Replacement Therapy on Functional Rescue and Longevity in Dystrophic Mice
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P2860
The transgenic expression of LARGE exacerbates the muscle phenotype of dystroglycanopathy mice.
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The transgenic expression of L ...... pe of dystroglycanopathy mice.
@en
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@nl
type
label
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@en
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@nl
prefLabel
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@en
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@nl
P2093
P2860
P356
P1476
The transgenic expression of L ...... pe of dystroglycanopathy mice.
@en
P2093
Attia Ashraf
Callum Parr
Charlotte Whitmore
Erica Lacey
Francesco Muntoni
Helen Booler
Kim E Wells
Manoli Kavishwar
Mark R Ackroyd
P2860
P304
P356
10.1093/HMG/DDT577
P577
2013-11-13T00:00:00Z