Utrophin: A Structural and Functional Comparison to Dystrophin

The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein

1988 • 1,501 citations

Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy

1995 • 954 citations

Three muscular dystrophies: Loss of cytoskeleton-extracellular matrix linkage

1995 • 829 citations

The structural and functional diversity of dystrophin

1993 • 632 citations

The WW domain of Yes-associated protein binds a proline-rich ligand that differs from the consensus established for Src homology 3-binding modules.

1995 • 556 citations

Failure of postsynaptic specialization to develop at neuromuscular junctions of rapsyn-deficient mice

1995 • 530 citations

Dystroglycan-α, a dystrophin-associated glycoprotein, is a functional agrin receptor

1994 • 509 citations

Mutations in the Dystrophin-Associated Protein γ-Sarcoglycan in Chromosome 13 Muscular Dystrophy

1995 • 497 citations

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

1992 • 489 citations

An autosomal transcript in skeletal muscle with homology to dystrophin

1989 • 478 citations

β–sarcoglycan: characterization and role in limb–girdle muscular dystrophy linked to 4q12

1995 • 448 citations

A Short-Term Trial of Butyrate to Stimulate Fetal-Globin-Gene Expression in the β-Globin Disorders

1993 • 443 citations

β–sarcoglycan (A3b) mutations cause autosomal recessive muscular dystrophy with loss of the sarcoglycan complex

1995 • 440 citations

Localization of dystrophin to postsynaptic regions of central nervous system cortical neurons

1990 • 413 citations

Rescue of the En-1 Mutant Phenotype by Replacement of En-1 with En-2

1995 • 402 citations

The WW domain: a signalling site in dystrophin?

1994 • 380 citations

Primary structure of dystrophin-related protein

1992 • 380 citations

A role for dystrophin-associated glycoproteins and utrophin in agrin-induced AChR clustering

1994 • 369 citations

Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle

1991 • 368 citations

Alternative splicing of human dystrophin mRNA generates isoforms at the carboxy terminus

1989 • 332 citations

Dystrophin–glycoprotein complex: Its role in the molecular pathogenesis of muscular dystrophies

1994 • 309 citations

Identification and purification of an agrin receptor from torpedo postsynaptic membranes: A heteromeric complex related to the dystroglycans

1994 • 307 citations

An alternative dystrophin transcript specific to peripheral nerve

1993 • 280 citations

Dystroglycan binds nerve and muscle agrin

1994 • 272 citations

Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle

1991 • 261 citations

Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines.

1991 • 258 citations

Dp140: a novel 140 kDa CNS transcript from the dystrophin locus

1995 • 258 citations

Expression of four alternative dystrophin transcripts in brain regions regulated by different promoters

1992 • 249 citations

Augmentation by Erythropoietin of the Fetal-Hemoglobin Response to Hydroxyurea in Sickle Cell Disease

1993 • 247 citations

SH3 Domain-mediated Interaction of Dystroglycan and Grb2

1995 • 247 citations

A novel dystrophin isoform is required for normal retinal electrophysiology

1995 • 240 citations

A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other nonmuscle tissues.

1992 • 234 citations

The subcellular distribution of dystrophin in mouse skeletal, cardiac, and smooth muscle.

1991 • 228 citations

Utrophin actin binding domain: analysis of actin binding and cellular targeting

1995 • 215 citations

Glycoprotein‐binding site of dystrophin is confined to the cysteine‐rich domain and the first half of the carboxy‐terminal domain

1992 • 210 citations

Rapsyn may function as a link between the acetylcholine receptor and the agrin-binding dystrophin-associated glycoprotein complex

1995 • 209 citations

Syntrophin binds to an alternatively spliced exon of dystrophin.

1995 • 207 citations

Muscular Dystrophies—Diseases of the Dystrophin-Glycoprotein Complex

1995 • 204 citations

ZZ and TAZ: new putative zinc fingers in dystrophin and other proteins

1996 • 196 citations

Increasing complexity of the dystrophin-associated protein complex.

1994 • 194 citations

The Role of Agrin in Synapse Formation

1995 • 181 citations

The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathies

1992 • 168 citations

Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice-prone region of the dystrophin COOH terminus.

1995 • 164 citations

Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy

1994 • 162 citations

The 87K postsynaptic membrane protein from torpedo is a protein-tyrosine kinase substrate homologous to dystrophin

1993 • 158 citations

Human and murine dystrophin mRNA transcripts are differentially expressed during skeletal muscle, heart, and brain development

1992 • 155 citations

Distal transcript of the dystrophin gene initiated from an alternative first exon and encoding a 75-kDa protein widely distributed in nonmuscle tissues.

1992 • 146 citations

Dystrophin-related protein, utrophin, in normal and dystrophic human fetal skeletal muscle

1993 • 144 citations

Dystrophin and utrophin: the missing links!

1995 • 142 citations

Localization of dystrophin relative to acetylcholine receptor domains in electric tissue and adult and cultured skeletal muscle.

1991 • 140 citations

Localization and Quantitation of the Chromosome 6-Encoded Dystrophin-Related Protein in Normal and Pathological Human Muscle

1993 • 139 citations

Different distributions of dystrophin and related proteins at nerve-muscle junctions

1992 • 131 citations

Exogenous Dp71 restores the levels of dystrophin associated proteins but does not alleviate muscle damage in mdx mice

1994 • 129 citations

Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse.

1991 • 127 citations

β2-Syntrophin

1994 • 127 citations

Characterization of a 4.8kb transcript from the Duchenne muscular dystrophy locus expressed in Schwannoma cells

1992 • 119 citations

Clustering and immobilization of acetylcholine receptors by the 43-kD protein: a possible role for dystrophin-related protein.

1993 • 107 citations

Construction of a 2.6-Mb contig in yeast artificial chromosomes spanning the human dystrophin gene using an STS-based approach

1992 • 104 citations

The utrophin and dystrophin genes share similarities in genomic structure

1993 • 102 citations

The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain.

1992 • 99 citations

WWP, a New Amino Acid Motif Present in Single or Multiple Copies in Various Proteins Including Dystrophin and the SH3-Binding Yes-Associated Protein YAP65

1994 • 98 citations

Coiled-coil regions in the carboxy-terminal domains of dystrophin and related proteins: potentials for protein-protein interactions

1995 • 96 citations

Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis

1994 • 90 citations

Reciprocal expression of dystrophin and utrophin in muscles of Duchenne muscular dystrophy patients, female DMD-carriers and control subjects

1993 • 83 citations

Expression of a dystrophin-related protein associated with the skeletal muscle cell membrane

1991 • 77 citations

A yeast artificial chromosome contig containing the complete Duchenne muscular dystrophy gene

1992 • 74 citations

Apo-dystrophin-3: a 2.2kb transcript from the DMD locus encoding the dystrophin glycoprotein binding site

1993 • 74 citations

Differential expression of dystrophin, utrophin and dystrophin‐associated proteins in peripheral nerve

1993 • 70 citations

Localisation of a dystrophin-related autosomal gene to 6q24 in man, and to mouse chromosome 10 in the region of the dystrophia muscularis (dy) locus

1990 • 69 citations

G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain.

1995 • 65 citations

Utrophin localization in normal and dystrophin-deficient heart.

1994 • 64 citations

Expression of the dystrophin‐related protein (utrophin) gene during mouse embryogenesis

1993 • 60 citations

Asymmetric distribution of dystrophin in developing and adult Torpedo marmorata electrocyte: evidence for its association with the acetylcholine receptor-rich membrane.

1990 • 58 citations

Reversible transcriptional activation of mdr1 by sodium butyrate treatment of human colon cancer cells.

1994 • 55 citations

Direct binding of Torpedo syntrophin to dystrophin and the 87 kDa dystrophin homologue

1995 • 55 citations

Characterisation of dystrophin during development of human skeletal muscle

1992 • 53 citations

Isobutyramide, an orally bioavailable butyrate analogue, stimulates fetal globin gene expression in vitro and in vivo

1994 • 53 citations

Dystrophin is a component of the subsynaptic membrane.

1991 • 52 citations

Association of Aciculin with Dystrophin and Utrophin

1995 • 50 citations

Localization of dystrophin gene transcripts during mouse embryogenesis.

1992 • 48 citations

Clustering of the acetylcholine receptor by the 43-kD protein: involvement of the zinc finger domain.

1993 • 45 citations

Augmentation of gamma-globin gene promoter activity by carboxylic acids and components of the human beta-globin locus control region

1994 • 42 citations

Antibody against the C-Terminal Portion of Dystrophin Crossreacts with the 400 kDa Protein in the Pia Mater of Dystrophin-Deficient mdx Mouse Brain1

1990 • 40 citations

Purification of dystrophin‐related protein (utrophin) from lung and its identification in pulmonary artery endothelial cells

1993 • 37 citations

Dystroglycan mRNA expression during normal and mdx mouse embryogenesis: A comparison with utrophin and the apo‐dystrophins

1995 • 37 citations

Calcium/calmodulin‐dependent regulation of the NH₂‐terminal F‐actin binding domain of utrophin

1995 • 36 citations

Dystrophin‐related protein is found in the central nervous system of mice at various developmental stages, especially at the postsynaptic membrane

1994 • 28 citations

Re-evaluation of the distributions of dystrophin and utrophin in sciatic nerve

1995 • 27 citations

Immunoblot analysis of dystrophin-related protein (DRP)

1993 • 18 citations

Expression of a dystrophin-like protein on the surface membrane of muscle cells in mdx mice.

1989 • 17 citations

Laminin β2 (S-Laminin): a New Player at the Synapse

1995 • 13 citations

A homologue of dystrophin is expressed at the blood vessel membrane of DMD and BMD patients: Immunological evidence

1992 • 11 citations