Dystrophin deficiency in Drosophila reduces lifespan and causes a dilated cardiomyopathy phenotype.

PubWeight™: 1.52‹?› | Rank: Top 4%

🔗 View Article (PMC 2840698)

Published in Aging Cell on January 23, 2008

Authors

Ouarda Taghli-Lamallem1, Takeshi Akasaka, Grant Hogg, Uri Nudel, David Yaffe, Jeffrey S Chamberlain, Karen Ocorr, Rolf Bodmer

Author Affiliations

1: Development and Aging Program, Burnham Institute for Medical Research, 10901 North Torrey Pines Rd, Building 7, Room 7125, La Jolla, Ca 92037, USA.

Articles citing this

High-fat-diet-induced obesity and heart dysfunction are regulated by the TOR pathway in Drosophila. Cell Metab (2010) 2.50

Genetic modifier screens reveal new components that interact with the Drosophila dystroglycan-dystrophin complex. PLoS One (2008) 1.49

Cardiac aging: from molecular mechanisms to significance in human health and disease. Antioxid Redox Signal (2012) 1.40

Transcription factor neuromancer/TBX20 is required for cardiac function in Drosophila with implications for human heart disease. Proc Natl Acad Sci U S A (2008) 1.36

d4eBP acts downstream of both dTOR and dFoxo to modulate cardiac functional aging in Drosophila. Aging Cell (2009) 1.31

A Drosophila model of high sugar diet-induced cardiomyopathy. PLoS Genet (2013) 1.28

Flightless flies: Drosophila models of neuromuscular disease. Ann N Y Acad Sci (2010) 1.28

Exercise-training in young Drosophila melanogaster reduces age-related decline in mobility and cardiac performance. PLoS One (2009) 1.23

A mighty small heart: the cardiac proteome of adult Drosophila melanogaster. PLoS One (2011) 1.13

SMAD signaling drives heart and muscle dysfunction in a Drosophila model of muscular dystrophy. Hum Mol Genet (2010) 1.10

Fluorescent labeling of Drosophila heart structures. J Vis Exp (2009) 1.07

Drosophila as a model to study cardiac aging. Exp Gerontol (2010) 1.03

Huntington's disease induced cardiac amyloidosis is reversed by modulating protein folding and oxidative stress pathways in the Drosophila heart. PLoS Genet (2013) 1.00

Sestrins at the crossroad between stress and aging. Aging (Albany NY) (2010) 0.98

The roles of the dystrophin-associated glycoprotein complex at the synapse. Mol Neurobiol (2009) 0.96

Chronic hypoxia impairs muscle function in the Drosophila model of Duchenne's muscular dystrophy (DMD). PLoS One (2010) 0.95

Characterization, design, and function of the mitochondrial proteome: from organs to organisms. J Proteome Res (2013) 0.94

Obesity and nutrient sensing TOR pathway in flies and vertebrates: Functional conservation of genetic mechanisms. Trends Endocrinol Metab (2011) 0.94

Partial loss of GATA factor Pannier impairs adult heart function in Drosophila. Hum Mol Genet (2009) 0.93

Affecting Rhomboid-3 function causes a dilated heart in adult Drosophila. PLoS Genet (2010) 0.93

Genetic elevation of sphingosine 1-phosphate suppresses dystrophic muscle phenotypes in Drosophila. Development (2012) 0.93

Serial examination of an inducible and reversible dilated cardiomyopathy in individual adult Drosophila. PLoS One (2009) 0.90

A dual role for integrin-linked kinase and β1-integrin in modulating cardiac aging. Aging Cell (2014) 0.90

New dystrophin/dystroglycan interactors control neuron behavior in Drosophila eye. BMC Neurosci (2011) 0.86

Hyperthermic seizures and aberrant cellular homeostasis in Drosophila dystrophic muscles. Sci Rep (2011) 0.86

Drosophila models of cardiac disease. Prog Mol Biol Transl Sci (2011) 0.85

Methods to assess Drosophila heart development, function and aging. Methods (2014) 0.85

Non-autonomous modulation of heart rhythm, contractility and morphology in adult fruit flies. Dev Biol (2009) 0.85

Modeling dilated cardiomyopathies in Drosophila. Trends Cardiovasc Med (2012) 0.84

Multiple measures of functionality exhibit progressive decline in a parallel, stochastic fashion in Drosophila Sod2 null mutants. Biogerontology (2009) 0.84

A method to measure myocardial calcium handling in adult Drosophila. Circ Res (2011) 0.83

Probing the polygenic basis of cardiomyopathies in Drosophila. J Cell Mol Med (2012) 0.82

Pygopus maintains heart function in aging Drosophila independently of canonical Wnt signaling. Circ Cardiovasc Genet (2013) 0.81

Cardiomyopathy is associated with ribosomal protein gene haplo-insufficiency in Drosophila melanogaster. Genetics (2011) 0.81

The role of pygopus in the differentiation of intracardiac valves in Drosophila. Genesis (2013) 0.80

Pentamidine rescues contractility and rhythmicity in a Drosophila model of myotonic dystrophy heart dysfunction. Dis Model Mech (2015) 0.80

The evolutionarily conserved RNA binding protein SMOOTH is essential for maintaining normal muscle function. Fly (Austin) (2009) 0.79

Complex genetic architecture of cardiac disease in a wild type inbred strain of Drosophila melanogaster. PLoS One (2013) 0.79

Sizing up models of heart failure: Proteomics from flies to humans. Proteomics Clin Appl (2014) 0.78

Aging and CaMKII alter intracellular Ca2+ transients and heart rhythm in Drosophila melanogaster. PLoS One (2014) 0.78

Mechanical and non-mechanical functions of Dystrophin can prevent cardiac abnormalities in Drosophila. Exp Gerontol (2013) 0.78

Silencing of drpr leads to muscle and brain degeneration in adult Drosophila. Am J Pathol (2014) 0.77

Genetic manipulation of cardiac ageing. J Physiol (2015) 0.77

The conserved WW-domain binding sites in Dystroglycan C-terminus are essential but partially redundant for Dystroglycan function. BMC Dev Biol (2009) 0.77

The NADPH metabolic network regulates human αB-crystallin cardiomyopathy and reductive stress in Drosophila melanogaster. PLoS Genet (2013) 0.76

Decreased myocardial expression of dystrophin and titin mRNA and protein in dilated cardiomyopathy: possibly an adverse effect of TNF-alpha. J Clin Immunol (2010) 0.76

Deletion of Siah-interacting protein gene in Drosophila causes cardiomyopathy. Mol Genet Genomics (2012) 0.76

Extracellular matrix downregulation in the Drosophila heart preserves contractile function and improves lifespan. Matrix Biol (2016) 0.75

Age-dependent electrical and morphological remodeling of the Drosophila heart caused by hERG/seizure mutations. PLoS Genet (2017) 0.75

Articles cited by this

The genome sequence of Drosophila melanogaster. Science (2000) 74.32

Targeted gene expression as a means of altering cell fates and generating dominant phenotypes. Development (1993) 58.26

The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell (1988) 10.75

Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc Natl Acad Sci U S A (1993) 7.18

The muscular dystrophies. Lancet (2002) 6.41

Multiplex detection of RNA expression in Drosophila embryos. Science (2004) 5.27

Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev (2002) 4.80

The gene tinman is required for specification of the heart and visceral muscles in Drosophila. Development (1993) 4.11

Requirement of MADS domain transcription factor D-MEF2 for muscle formation in Drosophila. Science (1995) 3.64

Dystrophin and mutations: one gene, several proteins, multiple phenotypes. Lancet Neurol (2003) 3.46

Animal models for muscular dystrophy show different patterns of sarcolemmal disruption. J Cell Biol (1997) 3.32

KCNQ potassium channel mutations cause cardiac arrhythmias in Drosophila that mimic the effects of aging. Proc Natl Acad Sci U S A (2007) 3.30

Drosophila, the golden bug, emerges as a tool for human genetics. Nat Rev Genet (2005) 3.17

Dystrophic heart failure blocked by membrane sealant poloxamer. Nature (2005) 3.16

Insulin regulation of heart function in aging fruit flies. Nat Genet (2004) 3.02

Evolution of the mdx mouse cardiomyopathy: physiological and morphological findings. Neuromuscul Disord (2004) 2.34

The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy. Int J Cardiol (1990) 2.29

Dystrophin-deficient mdx mice display a reduced life span and are susceptible to spontaneous rhabdomyosarcoma. FASEB J (2007) 2.22

Microdystrophin gene therapy of cardiomyopathy restores dystrophin-glycoprotein complex and improves sarcolemma integrity in the mdx mouse heart. Circulation (2003) 2.22

Drosophila as a model for the identification of genes causing adult human heart disease. Proc Natl Acad Sci U S A (2006) 2.22

Age-associated cardiac dysfunction in Drosophila melanogaster. Circ Res (2001) 2.20

Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure. Mol Ther (2007) 1.85

Myosin transducer mutations differentially affect motor function, myofibril structure, and the performance of skeletal and cardiac muscles. Mol Biol Cell (2007) 1.81

Early signals in cardiac development. Circ Res (2002) 1.73

Drosophila, an emerging model for cardiac disease. Gene (2004) 1.67

Ostia, the inflow tracts of the Drosophila heart, develop from a genetically distinct subset of cardial cells. Mech Dev (2001) 1.65

Prevention of dystrophin-deficient cardiomyopathy in twenty-one-month-old carrier mice by mosaic dystrophin expression or complementary dystrophin/utrophin expression. Circ Res (2007) 1.65

The held out wings (how) Drosophila gene encodes a putative RNA-binding protein involved in the control of muscular and cardiac activity. Development (1997) 1.58

Dissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy. EMBO J (2007) 1.58

Adeno-associated virus vector-mediated minidystrophin gene therapy improves dystrophic muscle contractile function in mdx mice. Hum Gene Ther (2002) 1.56

Human neurodegenerative disease modeling using Drosophila. Annu Rev Neurosci (2003) 1.53

Cardiomyopathy in dystrophin-deficient hearts is prevented by expression of a neuronal nitric oxide synthase transgene in the myocardium. Hum Mol Genet (2005) 1.53

Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex. J Cell Sci (2006) 1.51

Characterization of components of Z-bands in the fibrillar flight muscle of Drosophila melanogaster. J Cell Biol (1989) 1.45

Intracellular calcium handling in ventricular myocytes from mdx mice. Am J Physiol Heart Circ Physiol (2006) 1.45

Screening assays for heart function mutants in Drosophila. Biotechniques (2004) 1.43

Modeling human neurodegenerative diseases in Drosophila: on a wing and a prayer. Trends Genet (2000) 1.41

Perlecan and Dystroglycan act at the basal side of the Drosophila follicular epithelium to maintain epithelial organization. Development (2006) 1.39

Gene therapy of muscular dystrophy. Hum Mol Genet (2002) 1.38

Subtle neuromuscular defects in utrophin-deficient mice. J Cell Biol (1997) 1.37

Stable fetal cardiomyocyte grafts in the hearts of dystrophic mice and dogs. J Clin Invest (1995) 1.36

Steroid-dependent modification of Hox function drives myocyte reprogramming in the Drosophila heart. Development (2005) 1.36

Heart development in Drosophila and its relationship to vertebrates. Trends Cardiovasc Med (2011) 1.35

Stretch-sensitive channels in developing muscle cells from a mouse cell line. J Physiol (1990) 1.24

Dystrophin Dp71 is critical for the clustered localization of potassium channels in retinal glial cells. J Neurosci (2002) 1.23

The role of cell adhesion molecules in Drosophila heart morphogenesis: faint sausage, shotgun/DE-cadherin, and laminin A are required for discrete stages in heart development. Dev Biol (1999) 1.22

Role of nitric oxide in the pathogenesis of muscular dystrophies: a "two hit" hypothesis of the cause of muscle necrosis. Microsc Res Tech (2001) 1.21

Mutations in the Caenorhabditis elegans dystrophin-like gene dys-1 lead to hyperactivity and suggest a link with cholinergic transmission. Neurogenetics (1998) 1.18

Cardiac dysfunction with Becker muscular dystrophy. Am Heart J (1996) 1.16

Neuromancer Tbx20-related genes (H15/midline) promote cell fate specification and morphogenesis of the Drosophila heart. Dev Biol (2005) 1.12

Conservation of components of the dystrophin complex in Drosophila. FEBS Lett (2000) 1.11

Genetic suppression of phenotypes arising from mutations in dystrophin-related genes in Caenorhabditis elegans. Curr Biol (2000) 1.09

Conditional mutations in SERCA, the Sarco-endoplasmic reticulum Ca2+-ATPase, alter heart rate and rhythmicity in Drosophila. J Comp Physiol B (2005) 1.08

Identification of a zebrafish model of muscular dystrophy. Clin Exp Pharmacol Physiol (2004) 1.06

Dystrophin is required for appropriate retrograde control of neurotransmitter release at the Drosophila neuromuscular junction. J Neurosci (2006) 1.05

Cardiac abnormalities and skeletal muscle weakness in carriers of Duchenne and Becker muscular dystrophies and controls. Neuromuscul Disord (2001) 1.04

Developmental paradigms in heart disease: insights from tinman. Ann Med (2002) 1.02

The SLO-1 BK channel of Caenorhabditis elegans is critical for muscle function and is involved in dystrophin-dependent muscle dystrophy. J Mol Biol (2006) 1.02

The Drosophila muscle LIM protein, Mlp84B, is essential for cardiac function. J Exp Biol (2008) 1.01

Modulation of the cardiac pacemaker of Drosophila: cellular mechanisms. J Comp Physiol B (2002) 0.94

Embryonic expression patterns of the Drosophila dystrophin-associated glycoprotein complex orthologs. Gene Expr Patterns (2004) 0.93

Cardiac enhancer activity of the homeobox gene tinman depends on CREB consensus binding sites in Drosophila. Genesis (2000) 0.92

The dystrophin / utrophin homologues in Drosophila and in sea urchin. Gene (2001) 0.92

Decreased mAKAP, ryanodine receptor, and SERCA2a gene expression in mdx hearts. Biochem Biophys Res Commun (2003) 0.87

Expression and regulation of the dystrophin Purkinje promoter in human skeletal muscle, heart, and brain. Hum Genet (1996) 0.86

Cardiac involvement in muscular dystrophies: molecular mechanisms. Muscle Nerve (2005) 0.85

Stretch-induced activation of Ca(2+)-activated K(+) channels in mouse skeletal muscle fibers. Am J Physiol Cell Physiol (2000) 0.83

Tumour suppressors--a fly's perspective. Eur J Cancer (2003) 0.83

The Drosophila homologue of the dystrophin gene - introns containing promoters are the major contributors to the large size of the gene. FEBS Lett (2005) 0.82

Ultrastructural changes in the cardiomyopathy of dystrophic hamsters and mice. Tissue Cell (1988) 0.77

Articles by these authors

Systemic delivery of genes to striated muscles using adeno-associated viral vectors. Nat Med (2004) 5.59

Pericytes of human skeletal muscle are myogenic precursors distinct from satellite cells. Nat Cell Biol (2007) 5.50

Modular flexibility of dystrophin: implications for gene therapy of Duchenne muscular dystrophy. Nat Med (2002) 5.07

Sestrin as a feedback inhibitor of TOR that prevents age-related pathologies. Science (2010) 3.86

KCNQ potassium channel mutations cause cardiac arrhythmias in Drosophila that mimic the effects of aging. Proc Natl Acad Sci U S A (2007) 3.30

rAAV6-microdystrophin preserves muscle function and extends lifespan in severely dystrophic mice. Nat Med (2006) 3.13

Insulin regulation of heart function in aging fruit flies. Nat Genet (2004) 3.02

Dystrophins carrying spectrin-like repeats 16 and 17 anchor nNOS to the sarcolemma and enhance exercise performance in a mouse model of muscular dystrophy. J Clin Invest (2009) 2.89

Sarcolemma-localized nNOS is required to maintain activity after mild exercise. Nature (2008) 2.87

Efficient transduction of skeletal muscle using vectors based on adeno-associated virus serotype 6. Mol Ther (2004) 2.84

Sustained AAV-mediated dystrophin expression in a canine model of Duchenne muscular dystrophy with a brief course of immunosuppression. Mol Ther (2007) 2.76

High-fat-diet-induced obesity and heart dysfunction are regulated by the TOR pathway in Drosophila. Cell Metab (2010) 2.50

A new method for detection and quantification of heartbeat parameters in Drosophila, zebrafish, and embryonic mouse hearts. Biotechniques (2009) 2.30

A global in vivo Drosophila RNAi screen identifies NOT3 as a conserved regulator of heart function. Cell (2010) 2.27

Microdystrophin gene therapy of cardiomyopathy restores dystrophin-glycoprotein complex and improves sarcolemma integrity in the mdx mouse heart. Circulation (2003) 2.22

Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors. Nat Biotechnol (2005) 2.16

Adeno-associated virus-mediated microdystrophin expression protects young mdx muscle from contraction-induced injury. Mol Ther (2005) 2.08

Antioxidants protect PINK1-dependent dopaminergic neurons in Drosophila. Proc Natl Acad Sci U S A (2006) 2.08

Dynamics of Cux2 expression suggests that an early pool of SVZ precursors is fated to become upper cortical layer neurons. Cereb Cortex (2004) 1.99

Activated FOXO-mediated insulin resistance is blocked by reduction of TOR activity. Cell Metab (2006) 1.99

Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure. Mol Ther (2007) 1.85

BIN1 is reduced and Cav1.2 trafficking is impaired in human failing cardiomyocytes. Heart Rhythm (2011) 1.85

Myosin transducer mutations differentially affect motor function, myofibril structure, and the performance of skeletal and cardiac muscles. Mol Biol Cell (2007) 1.81

In vivo cardiac reprogramming contributes to zebrafish heart regeneration. Nature (2013) 1.79

Immunity to adeno-associated virus-mediated gene transfer in a random-bred canine model of Duchenne muscular dystrophy. Hum Gene Ther (2007) 1.70

Drosophila, an emerging model for cardiac disease. Gene (2004) 1.67

Design of tissue-specific regulatory cassettes for high-level rAAV-mediated expression in skeletal and cardiac muscle. Mol Ther (2007) 1.65

The value of mammalian models for duchenne muscular dystrophy in developing therapeutic strategies. Curr Top Dev Biol (2008) 1.62

APJ acts as a dual receptor in cardiac hypertrophy. Nature (2012) 1.62

Dissecting muscle and neuronal disorders in a Drosophila model of muscular dystrophy. EMBO J (2007) 1.58

Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex. J Cell Sci (2006) 1.51

The ATP-sensitive potassium (KATP) channel-encoded dSUR gene is required for Drosophila heart function and is regulated by tinman. Proc Natl Acad Sci U S A (2006) 1.51

Functional correction of adult mdx mouse muscle using gutted adenoviral vectors expressing full-length dystrophin. Proc Natl Acad Sci U S A (2002) 1.47

Emergent dilated cardiomyopathy caused by targeted repair of dystrophic skeletal muscle. Mol Ther (2008) 1.47

Recombinant adeno-associated virus transduction and integration. Mol Ther (2008) 1.44

Genetic control of heart function and aging in Drosophila. Trends Cardiovasc Med (2007) 1.43

Screening assays for heart function mutants in Drosophila. Biotechniques (2004) 1.43

Functional deficits in nNOSmu-deficient skeletal muscle: myopathy in nNOS knockout mice. PLoS One (2008) 1.42

Slit and Robo control cardiac cell polarity and morphogenesis. Curr Biol (2005) 1.41

Long-term persistence of donor nuclei in a Duchenne muscular dystrophy patient receiving bone marrow transplantation. J Clin Invest (2002) 1.41

Prehospital Transfer Pathway and Mortality in Patients Undergoing Primary Percutaneous Coronary Intervention. Circ J (2015) 1.39

Dispensable role of Drosophila ortholog of LRRK2 kinase activity in survival of dopaminergic neurons. Mol Neurodegener (2008) 1.37

Cardioblast-intrinsic Tinman activity controls proper diversification and differentiation of myocardial cells in Drosophila. Development (2006) 1.36

Transcription factor neuromancer/TBX20 is required for cardiac function in Drosophila with implications for human heart disease. Proc Natl Acad Sci U S A (2008) 1.36

Prevention of muscle aging by myofiber-associated satellite cell transplantation. Sci Transl Med (2010) 1.36

Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/utrophin-deficient mice. Mol Ther (2008) 1.34

d4eBP acts downstream of both dTOR and dFoxo to modulate cardiac functional aging in Drosophila. Aging Cell (2009) 1.31

Systemic microdystrophin gene delivery improves skeletal muscle structure and function in old dystrophic mdx mice. Mol Ther (2008) 1.29

Emerging strategies for cell and gene therapy of the muscular dystrophies. Expert Rev Mol Med (2009) 1.29

A Drosophila model of high sugar diet-induced cardiomyopathy. PLoS Genet (2013) 1.28

Targeted inactivation of dystrophin gene product Dp71: phenotypic impact in mouse retina. Hum Mol Genet (2003) 1.27

Involvement of the Polycomb-group gene Ring1B in the specification of the anterior-posterior axis in mice. Development (2002) 1.26

Viral-mediated gene therapy for the muscular dystrophies: successes, limitations and recent advances. Biochim Biophys Acta (2006) 1.25

Myogenic cells fates are antagonized by Notch only in asymmetric lineages of the Drosophila heart, with or without cell division. Development (2003) 1.25

Viral vectors for gene transfer of micro-, mini-, or full-length dystrophin. Neuromuscul Disord (2002) 1.24

Gene therapy of mdx mice with large truncated dystrophins generated by recombination using rAAV6. Mol Ther (2010) 1.21

Images in cardiovascular medicine: in vivo imaging of the adult Drosophila melanogaster heart with real-time optical coherence tomography. Circulation (2006) 1.21

Cell-lineage regulated myogenesis for dystrophin replacement: a novel therapeutic approach for treatment of muscular dystrophy. Hum Mol Genet (2008) 1.19

An ex vivo gene therapy approach to treat muscular dystrophy using inducible pluripotent stem cells. Nat Commun (2013) 1.17

Somatic instability of the expanded CTG triplet repeat in myotonic dystrophy type 1 is a heritable quantitative trait and modifier of disease severity. Hum Mol Genet (2012) 1.16

Phospholipid homeostasis regulates lipid metabolism and cardiac function through SREBP signaling in Drosophila. Genes Dev (2011) 1.16

Gene therapy strategies for Duchenne muscular dystrophy utilizing recombinant adeno-associated virus vectors. Mol Ther (2005) 1.15

Semi-automated Optical Heartbeat Analysis of small hearts. J Vis Exp (2009) 1.15

Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin. J Cell Sci (2010) 1.14

Gene and cell-mediated therapies for muscular dystrophy. Muscle Nerve (2013) 1.14

Successful regional delivery and long-term expression of a dystrophin gene in canine muscular dystrophy: a preclinical model for human therapies. Mol Ther (2012) 1.13

Evaluation of vascular delivery methodologies to enhance rAAV6-mediated gene transfer to canine striated musculature. Mol Ther (2009) 1.13

A mighty small heart: the cardiac proteome of adult Drosophila melanogaster. PLoS One (2011) 1.13

AmphiNk2-tin, an amphioxus homeobox gene expressed in myocardial progenitors: insights into evolution of the vertebrate heart. Dev Biol (2003) 1.13

rAAV6-microdystrophin rescues aberrant Golgi complex organization in mdx skeletal muscles. Traffic (2007) 1.13

Transcriptional integration of competence modulated by mutual repression generates cell-type specificity within the cardiogenic mesoderm. Dev Biol (2002) 1.12

Visualizing the beating heart in Drosophila. J Vis Exp (2009) 1.12

Neuromancer Tbx20-related genes (H15/midline) promote cell fate specification and morphogenesis of the Drosophila heart. Dev Biol (2005) 1.12

Immune responses to AAV in canine muscle monitored by cellular assays and noninvasive imaging. Mol Ther (2009) 1.11

A highly functional mini-dystrophin/GFP fusion gene for cell and gene therapy studies of Duchenne muscular dystrophy. Hum Mol Genet (2006) 1.11

Regeneration and transdifferentiation potential of muscle-derived stem cells propagated as myospheres. Stem Cells (2006) 1.10

Expression of Dp260 in muscle tethers the actin cytoskeleton to the dystrophin-glycoprotein complex and partially prevents dystrophy. Hum Mol Genet (2002) 1.10

Age-related cardiac disease model of Drosophila. Mech Ageing Dev (2006) 1.10

Phenotypic improvement of dystrophic muscles by rAAV/microdystrophin vectors is augmented by Igf1 codelivery. Mol Ther (2005) 1.10

Follistatin-mediated skeletal muscle hypertrophy is regulated by Smad3 and mTOR independently of myostatin. J Cell Biol (2012) 1.10

Inhibitory control over Ca(2+) sparks via mechanosensitive channels is disrupted in dystrophin deficient muscle but restored by mini-dystrophin expression. PLoS One (2008) 1.08

The polyproline site in hinge 2 influences the functional capacity of truncated dystrophins. PLoS Genet (2010) 1.08

Gata factor Pannier is required to establish competence for heart progenitor formation. Development (2003) 1.08