Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin α2-deficient mice

Dystrophin: The protein product of the duchenne muscular dystrophy locus

1987 • 4,477 citations

The Molecular Basis of Muscular Dystrophy in the mdx Mouse: a Point Mutation

1989 • 1,171 citations

The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy

1991 • 892 citations

Utrophin-Dystrophin-Deficient Mice as a Model for Duchenne Muscular Dystrophy

1997 • 710 citations

Skeletal and Cardiac Myopathies in Mice Lacking Utrophin and Dystrophin: A Model for Duchenne Muscular Dystrophy

1997 • 625 citations

Mutations in the laminin α2–chain gene (LAMA2) cause merosin–deficient congenital muscular dystrophy

1995 • 615 citations

Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice

1999 • 571 citations

Clinical trial in duchenne dystrophy. I. The design of the protocol

1981 • 523 citations

Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle

1992 • 489 citations

Animal Models for Muscular Dystrophy Show Different Patterns of Sarcolemmal Disruption

1997 • 482 citations

Congenital muscular dystrophy with merosin deficiency.

1994 • 449 citations

Distribution and Function of Laminins in the Neuromuscular System of Developing, Adult, and Mutant Mice

1997 • 433 citations

Clinical investigation in duchenne dystrophy: 2. Determination of the “power” of therapeutic trials based on the natural history

1983 • 354 citations

Murine muscular dystrophy caused by a mutation in the laminin α2 (Lama2) gene

1994 • 346 citations

Role for α-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies

1999 • 309 citations

Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus.

1994 • 299 citations

Muscular dystrophy in the mdx mouse: Histopathology of the soleus and extensor digitorum longus muscles

1987 • 282 citations

Overexpression of dystrophin in transgenic mdx mice eliminates dystrophic symptoms without toxicity

1993 • 281 citations

Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice

1995 • 277 citations

Maturation and Maintenance of the Neuromuscular Synapse

2000 • 276 citations

Abnormalities of peripheral nerves in murine muscular dystrophy

1973 • 259 citations

Defective muscle basement membrane and lack of M-laminin in the dystrophic dy/dy mouse.

1994 • 254 citations

Laminin α2 chain‐null mutant mice by targeted disruption of the Lama2 gene: a new model of merosin (laminin 2)‐deficient congenital muscular dystrophy

1997 • 252 citations

Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice

1997 • 241 citations

Subtle Neuromuscular Defects in Utrophin-deficient Mice

1997 • 216 citations

Fiber regeneration is not persistent in dystrophic (mdx) mouse skeletal muscle

1991 • 200 citations

Localization of merosin-negative congenital muscular dystrophy to chromosome 6q2 by homozygosity mapping

1994 • 195 citations

Phenotype of dystrophinopathy in old MDX mice

1995 • 191 citations

Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models.

1998 • 185 citations

Forced expression of dystrophin deletion constructs reveals structure-function correlations.

1996 • 180 citations

Clinical phenotype in congenital muscular dystrophy: correlation with expression of merosin in skeletal

1995 • 180 citations

Structure and function of the neuromuscular junction in young adultmdx mice

1991 • 179 citations

Pre-clinical screening of drugs using the mdx mouse

2000 • 172 citations

Apoptosis, DNA damage and ubiquitin expression in normal and mdx muscle fibers after exercise

1995 • 170 citations

Identification of a novel mutant transcript of laminin α2 chain gene responsible for muscular dystrophy and dysmyelination in dy^2J mice

1995 • 170 citations

Motor dysfunction in a mouse model for Down syndrome

1999 • 166 citations

Clinical Investigation of Duchenne Muscular Dystrophy

1987 • 153 citations

Dystroglycan is a binding protein of laminin and merosin in peripheral nerve

1994 • 151 citations

Laminin α2 muscular dystrophy

1998 • 116 citations

Congenital muscular dystrophy with primary laminin α2 (merosin) deficiency presenting as inflammatory myopathy

1996 • 112 citations

Expression of laminin subunits in congenital muscular dystrophy

1995 • 108 citations

A common mutation (ε1267delG) in congenital myasthenic patients of Gypsy ethnic origin

1999 • 106 citations

Beneficial effects of insulin-like growth factor-I on wobbler mouse motoneuron disease

1995 • 93 citations

Genetically determined defect of Schwann cell basement membrane in dystrophic mouse

1975 • 92 citations

How does dystrophin deficiency lead to muscle degeneration? — Evidence from the MDX mouse

1995 • 86 citations

Peripheral nerve involvement in merosin-deficient congenital muscular dystrophy and dy mouse

1997 • 85 citations

Distribution of ten laminin chains in dystrophic and regenerating muscles

1999 • 80 citations

Quantitative measurement of muscle strength in the mouse

1995 • 78 citations

Merosin/laminin-2 and muscular dystrophy

1996 • 78 citations

A simple recording grip strength device

1978 • 68 citations

Neuromuscular transmission in the mdx mouse

1990 • 68 citations

Somatosensory and Visual Evoked Potentials in Congenital Muscular Dystrophy: Correlation with MRI Changes and Muscle Merosin Status

1995 • 64 citations

Strength and endurance in the therapeutic evaluation of prednisolone-treated MDX mice.

1993 • 60 citations

Congenital muscular dystrophy

1996 • 60 citations

Abnormal merosin in adults. A new form of late onset muscular dystrophy not linked to chromosome 6q2

1998 • 59 citations

Neuronal nitric oxide synthase inhibitor, 7-nitroindazole, delays motor dysfunction and spinal motoneuron degeneration in the wobbler mouse

1998 • 57 citations

The Childhood Muscular Dystrophies: Diseases Sharing a Common Pathogenesis of Membrane Instability

1995 • 48 citations

Behavioral Performance of Swiss Webster Mice Exposed to Excess Dietary Aluminum during Development or during Development and as Adults

1995 • 46 citations

Dystrophy and myogenesis inmdx diaphragm muscle

1998 • 45 citations

The role of immunocytochemistry in congenital myopathies

1998 • 42 citations

An ultrastructural study of degeneration and necrosis of muscle in the dystrophic mouse

1970 • 40 citations

Congenital muscular dystrophy syndromes distinguished by alkaline and acid phosphatase, merosin, and dystrophin staining

1996 • 26 citations

Differential labelling of laminin alpha 2 in muscle and neural tissue of dy/dy mice: are there isoforms of the laminin alpha 2 chain?

1998 • 23 citations

Neuromuscular transmission in dystrophic mice

1977 • 22 citations

Morphological and functional study of extensor digitorum longus muscle regeneration after iterative crush lesions in mdx mouse

1995 • 18 citations

Long‐term neuroprotective effects of glycosaminoglycans–IGF‐I cotreatment in the motor neuron degeneration (mnd ) mutant mouse

1999 • 17 citations

Comparison of dy and dy2J, Two Alleles Expressing Forms of Muscular Dystrophy in the Mouse

1976 • 16 citations

Merosin and Laminin.

1990 • 15 citations

A comparative freeze‐fracture study of plasma membrane of dystrophic skeletal muscles in dy/dy mice with merosin (laminin 2) deficiency and mdx mice with dystrophin deficiency

1997 • 9 citations

Murine Muscular Dystrophy

1979 • 2 citations