Published in Cell on August 20, 1999
MG53 nucleates assembly of cell membrane repair machinery. Nat Cell Biol (2008) 3.24
Gene expression comparison of biopsies from Duchenne muscular dystrophy (DMD) and normal skeletal muscle. Proc Natl Acad Sci U S A (2002) 2.60
Functional muscle ischemia in neuronal nitric oxide synthase-deficient skeletal muscle of children with Duchenne muscular dystrophy. Proc Natl Acad Sci U S A (2000) 2.49
Lack of type XV collagen causes a skeletal myopathy and cardiovascular defects in mice. Proc Natl Acad Sci U S A (2001) 2.26
Drosophila as a model for the identification of genes causing adult human heart disease. Proc Natl Acad Sci U S A (2006) 2.22
Episodic coronary artery vasospasm and hypertension develop in the absence of Sur2 K(ATP) channels. J Clin Invest (2002) 2.11
Mutations in the human delta-sarcoglycan gene in familial and sporadic dilated cardiomyopathy. J Clin Invest (2000) 1.91
Dysferlin-mediated membrane repair protects the heart from stress-induced left ventricular injury. J Clin Invest (2007) 1.88
Age-dependent effect of myostatin blockade on disease severity in a murine model of limb-girdle muscular dystrophy. Am J Pathol (2006) 1.64
Genetic analyses of integrin signaling. Cold Spring Harb Perspect Biol (2011) 1.46
Challenges and opportunities in dystrophin-deficient cardiomyopathy gene therapy. Hum Mol Genet (2006) 1.44
Exercise-induced left ventricular systolic dysfunction in women heterozygous for dystrophinopathy. J Am Soc Echocardiogr (2010) 1.43
Prevention of cardiomyopathy in mouse models lacking the smooth muscle sarcoglycan-sarcospan complex. J Clin Invest (2001) 1.41
Dilated cardiomyopathy in mice deficient for the lysosomal cysteine peptidase cathepsin L. Proc Natl Acad Sci U S A (2002) 1.37
Transplanted hematopoietic stem cells demonstrate impaired sarcoglycan expression after engraftment into cardiac and skeletal muscle. J Clin Invest (2004) 1.23
Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects. Expert Rev Mol Med (2009) 1.22
Induction of pulmonary hypertension by an angiopoietin 1/TIE2/serotonin pathway. Proc Natl Acad Sci U S A (2003) 1.17
Smooth muscle cell-extrinsic vascular spasm arises from cardiomyocyte degeneration in sarcoglycan-deficient cardiomyopathy. J Clin Invest (2004) 1.14
The dystrophin complex controls bk channel localization and muscle activity in Caenorhabditis elegans. PLoS Genet (2009) 1.12
Genome-wide association study of anthropometric traits in Korcula Island, Croatia. Croat Med J (2009) 1.10
SMAD signaling drives heart and muscle dysfunction in a Drosophila model of muscular dystrophy. Hum Mol Genet (2010) 1.10
Absence of dystrophin in mice reduces NO-dependent vascular function and vascular density: total recovery after a treatment with the aminoglycoside gentamicin. Arterioscler Thromb Vasc Biol (2004) 1.08
Autophagic cardiomyocyte death in cardiomyopathic hamsters and its prevention by granulocyte colony-stimulating factor. Am J Pathol (2006) 1.08
The genetic basis of cardiac function: dissection by zebrafish (Danio rerio) screens. Philos Trans R Soc Lond B Biol Sci (2000) 1.05
Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex. Hum Mol Genet (2011) 1.05
Large-scale candidate gene analysis of HDL particle features. PLoS One (2011) 1.04
Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage. Circ Res (2009) 1.03
Genetic disruption of calcineurin improves skeletal muscle pathology and cardiac disease in a mouse model of limb-girdle muscular dystrophy. J Biol Chem (2007) 1.00
Distinct pathophysiological mechanisms of cardiomyopathy in hearts lacking dystrophin or the sarcoglycan complex. FASEB J (2011) 0.99
Sarcospan-deficient mice maintain normal muscle function. Mol Cell Biol (2000) 0.98
Rescue of hereditary form of dilated cardiomyopathy by rAAV-mediated somatic gene therapy: amelioration of morphological findings, sarcolemmal permeability, cardiac performances, and the prognosis of TO-2 hamsters. Proc Natl Acad Sci U S A (2002) 0.97
Progress in gene therapy of dystrophic heart disease. Gene Ther (2012) 0.96
Young MLP deficient mice show diastolic dysfunction before the onset of dilated cardiomyopathy. J Mol Cell Cardiol (2005) 0.95
Cardiac disease in transgenic mice expressing human immunodeficiency virus-1 nef in cells of the immune system. Am J Pathol (2002) 0.94
Myocardial oxidative stress, osteogenic phenotype, and energy metabolism are differentially involved in the initiation and early progression of delta-sarcoglycan-null cardiomyopathy. Mol Cell Biochem (2008) 0.94
Delta-sarcoglycan gene therapy halts progression of cardiac dysfunction, improves respiratory failure, and prolongs life in myopathic hamsters. Circ Heart Fail (2010) 0.93
Overexpression of Galgt2 reduces dystrophic pathology in the skeletal muscles of alpha sarcoglycan-deficient mice. Am J Pathol (2009) 0.92
Gene transfer establishes primacy of striated vs. smooth muscle sarcoglycan complex in limb-girdle muscular dystrophy. Proc Natl Acad Sci U S A (2003) 0.91
Secondary coronary artery vasospasm promotes cardiomyopathy progression. Am J Pathol (2004) 0.91
δ-Sarcoglycan-deficient muscular dystrophy: from discovery to therapeutic approaches. Skelet Muscle (2011) 0.90
Vascular dysfunction in idiopathic dilated cardiomyopathy. Nat Rev Cardiol (2009) 0.89
Flt-1 haploinsufficiency ameliorates muscular dystrophy phenotype by developmentally increased vasculature in mdx mice. Hum Mol Genet (2010) 0.88
Myocardial cell damage in Duchenne muscular dystrophy. Pediatr Cardiol (2003) 0.88
Long-term survival of transplanted stem cells in immunocompetent mice with muscular dystrophy. Am J Pathol (2008) 0.86
Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy. Mitochondrion (2011) 0.86
Sarcolemma instability during mechanical activity in Largemyd cardiac myocytes with loss of dystroglycan extracellular matrix receptor function. Hum Mol Genet (2011) 0.86
S151A δ-sarcoglycan mutation causes a mild phenotype of cardiomyopathy in mice. Eur J Hum Genet (2013) 0.86
A common disease-associated missense mutation in alpha-sarcoglycan fails to cause muscular dystrophy in mice. Hum Mol Genet (2008) 0.86
Rodent models of heart failure: an updated review. Heart Fail Rev (2013) 0.85
Sarcospan: a small protein with large potential for Duchenne muscular dystrophy. Skelet Muscle (2013) 0.85
Heterogeneous abnormalities of in-vivo left ventricular calcium influx and function in mouse models of muscular dystrophy cardiomyopathy. J Cardiovasc Magn Reson (2013) 0.85
Beta-blockers, left and right ventricular function, and in-vivo calcium influx in muscular dystrophy cardiomyopathy. PLoS One (2013) 0.84
A novel isoform of delta-sarcoglycan is localized at the sarcoplasmic reticulum of mouse skeletal muscle. Biochem Biophys Res Commun (2005) 0.84
Mutation of delta-sarcoglycan is associated with Ca(2+) -dependent vascular remodeling in the Syrian hamster. Am J Pathol (2007) 0.84
Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. PLoS One (2008) 0.84
Animal models of muscular dystrophy. Prog Mol Biol Transl Sci (2012) 0.83
Structural and functional analysis of the sarcoglycan-sarcospan subcomplex. Exp Cell Res (2006) 0.83
Quantitative trait loci for exercise training responses in FVB/NJ and C57BL/6J mice. Physiol Genomics (2009) 0.82
The sarcoglycan-sarcospan complex localization in mouse retina is independent from dystrophins. Neurosci Res (2005) 0.81
Muscle fatigue, nNOS and muscle fiber atrophy in limb girdle muscular dystrophy. Acta Myol (2014) 0.81
Alpha7beta1 integrin does not alleviate disease in a mouse model of limb girdle muscular dystrophy type 2F. Am J Pathol (2007) 0.80
Magnetic resonance imaging assessment of cardiac dysfunction in δ-sarcoglycan null mice. Neuromuscul Disord (2010) 0.80
Does delta-sarcoglycan-associated autosomal-dominant cardiomyopathy exist? Eur J Hum Genet (2009) 0.80
ECM-related gene expression profile in vascular smooth muscle cells from human saphenous vein and internal thoracic artery. J Cardiothorac Surg (2013) 0.79
Chronic oral administration of Ang-(1-7) improves skeletal muscle, autonomic and locomotor phenotypes in muscular dystrophy. Clin Sci (Lond) (2014) 0.79
Equal force recovery in dysferlin-deficient and wild-type muscles following saponin exposure. J Biomed Biotechnol (2011) 0.79
What do mouse models of muscular dystrophy tell us about the DAPC and its components? Int J Exp Pathol (2014) 0.79
Matrix metalloproteinases: not-so-innocent bystanders in heart failure. J Clin Invest (2000) 0.78
Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy-Associated Cardiomyopathy. J Am Heart Assoc (2015) 0.78
Morphological and Molecular Characterization of Human Dermal Lymphatic Collectors. PLoS One (2016) 0.78
Hemodynamic alterations in the coronary circulation of cardiomyopathic hamsters: age and Ang II-dependent mechanisms. J Card Fail (2009) 0.77
Therapeutic possibilities in the autosomal recessive limb-girdle muscular dystrophies. Neurotherapeutics (2008) 0.77
Altered calcium pump and secondary deficiency of gamma-sarcoglycan and microspan in sarcoplasmic reticulum membranes isolated from delta-sarcoglycan knockout mice. Cell Calcium (2010) 0.77
Increased neointimal thickening in dystrophin-deficient mdx mice. PLoS One (2012) 0.77
Genetic variation at the delta-sarcoglycan (SGCD) locus elevates heritable sympathetic nerve activity in human twin pairs. J Neurochem (2013) 0.77
Sarcoglycan, the heart, and skeletal muscles: new treatment, old drug? J Clin Invest (2001) 0.77
Molecular Signatures of Membrane Protein Complexes Underlying Muscular Dystrophy. Mol Cell Proteomics (2016) 0.76
Fusion of bone marrow-derived stem cells with striated muscle may not be sufficient to activate muscle genes. J Clin Invest (2004) 0.76
Modulation of Vascular ACE by Oxidative Stress in Young Syrian Cardiomyopathic Hamsters: Therapeutic Implications. J Clin Med (2016) 0.75
Angiotensin-dependent autonomic dysregulation precedes dilated cardiomyopathy in a mouse model of muscular dystrophy. Exp Physiol (2015) 0.75
(-)-Epicatechin improves mitochondrial-related protein levels and ameliorates oxidative stress in dystrophic δ-sarcoglycan null mouse striated muscle. FEBS J (2014) 0.75
Mendelian forms of structural cardiovascular disease. Curr Cardiol Rep (2013) 0.75
Myocardial Contractile Dysfunction Is Present without Histopathology in a Mouse Model of Limb-Girdle Muscular Dystrophy-2F and Is Prevented after Claudin-5 Virotherapy. Front Physiol (2016) 0.75
Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex. Anat Rec (Hoboken) (2014) 0.75
Collagen VI deficiency reduces muscle pathology, but does not improve muscle function, in the γ-sarcoglycan-null mouse. Hum Mol Genet (2016) 0.75
Natural disease history of mouse models for limb girdle muscular dystrophy types 2D and 2F. PLoS One (2017) 0.75
Linkage relationship of a cloned DNA sequence on the short arm of the X chromosome to Duchenne muscular dystrophy. Nature (1982) 11.57
Linkage analysis of two cloned DNA sequences flanking the Duchenne muscular dystrophy locus on the short arm of the human X chromosome. Nucleic Acids Res (1983) 8.92
Localization of cystic fibrosis locus to human chromosome 7cen-q22. Nature (1985) 7.77
A mutation in the interferon-gamma-receptor gene and susceptibility to mycobacterial infection. N Engl J Med (1996) 7.66
Effect of nesiritide in patients with acute decompensated heart failure. N Engl J Med (2011) 7.62
Membrane organization of the dystrophin-glycoprotein complex. Cell (1991) 6.64
Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix. Nature (1992) 6.37
The hydrophobic moment detects periodicity in protein hydrophobicity. Proc Natl Acad Sci U S A (1984) 6.36
Structural evidence for direct interaction between the molecular components of the transverse tubule/sarcoplasmic reticulum junction in skeletal muscle. J Cell Biol (1988) 6.30
Cloning of a representative genomic library of the human X chromosome after sorting by flow cytometry. Nature (1981) 6.18
Identification of alpha-dystroglycan as a receptor for lymphocytic choriomeningitis virus and Lassa fever virus. Science (1998) 5.83
A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin. J Cell Biol (1993) 5.73
Analysis of the beta-delta-globin gene loci in normal and Hb Lepore DNA: direct determination of gene linkage and intergene distance. Cell (1978) 5.30
Inducible, transferable resistance to vancomycin in Enterococcus faecalis A256. Antimicrob Agents Chemother (1989) 5.28
Novel inhibitors of cytokine-induced IkappaBalpha phosphorylation and endothelial cell adhesion molecule expression show anti-inflammatory effects in vivo. J Biol Chem (1997) 5.14
Chromosome 4q DNA rearrangements associated with facioscapulohumeral muscular dystrophy. Nat Genet (1992) 5.12
The helical hydrophobic moment: a measure of the amphiphilicity of a helix. Nature (1982) 5.01
Simple non-invasive method to obtain DNA for gene analysis. Lancet (1988) 4.96
Progress towards construction of a total restriction fragment map of a human chromosome. Nucleic Acids Res (1987) 4.68
Purified ryanodine receptor from rabbit skeletal muscle is the calcium-release channel of sarcoplasmic reticulum. J Gen Physiol (1988) 4.56
Cross-resistance to nalidixic acid, trimethoprim, and chloramphenicol associated with alterations in outer membrane proteins of Klebsiella, Enterobacter, and Serratia. J Infect Dis (1985) 4.52
Efficacy of superovulation and intrauterine insemination in the treatment of infertility. National Cooperative Reproductive Medicine Network. N Engl J Med (1999) 4.42
Calcium channel beta-subunit binds to a conserved motif in the I-II cytoplasmic linker of the alpha 1-subunit. Nature (1994) 4.34
The naming of voltage-gated calcium channels. Neuron (1994) 4.31
Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle. Nature (1990) 4.23
Genetic mapping of the human X chromosome by using restriction fragment length polymorphisms. Proc Natl Acad Sci U S A (1984) 4.22
Velo-cardio-facial syndrome associated with chromosome 22 deletions encompassing the DiGeorge locus. Lancet (1992) 4.07
Sperm morphology, motility, and concentration in fertile and infertile men. N Engl J Med (2001) 3.97
Cardiac hypertrophy is not a required compensatory response to short-term pressure overload. Circulation (2000) 3.82
Dissemination of the novel plasmid-mediated beta-lactamase CTX-1, which confers resistance to broad-spectrum cephalosporins, and its inhibition by beta-lactamase inhibitors. Antimicrob Agents Chemother (1988) 3.57
Quality-of-life changes and hearing impairment. A randomized trial. Ann Intern Med (1990) 3.52
Immunosuppression and resultant viral persistence by specific viral targeting of dendritic cells. J Exp Med (2000) 3.50
Sequence and expression of mRNAs encoding the alpha 1 and alpha 2 subunits of a DHP-sensitive calcium channel. Science (1988) 3.38
Animal models for muscular dystrophy show different patterns of sarcolemmal disruption. J Cell Biol (1997) 3.32
The mouse stargazer gene encodes a neuronal Ca2+-channel gamma subunit. Nat Genet (1998) 3.31
The mammalian sodium channel BNC1 is required for normal touch sensation. Nature (2000) 3.30
Muscular dystrophy and neuronal migration disorder caused by mutations in a glycosyltransferase, POMGnT1. Dev Cell (2001) 3.29
Purified ryanodine receptor from skeletal muscle sarcoplasmic reticulum is the Ca2+-permeable pore of the calcium release channel. J Biol Chem (1987) 3.25
Requirement for the leukocyte-specific adapter protein SLP-76 for normal T cell development. Science (1998) 3.18
Plasmid-mediated beta-lactamase (TEM-7) involved in resistance to ceftazidime and aztreonam. Rev Infect Dis (1988) 3.12
Nomenclature of voltage-gated calcium channels. Neuron (2000) 3.09
Psychological and social consequences of community carrier screening programme for cystic fibrosis. Lancet (1992) 3.06
The severe form of alpha thalassaemia is caused by a haemoglobin gene deletion. Nature (1974) 3.04
Patterns of polymorphism and linkage disequilibrium for cystic fibrosis. Genomics (1987) 3.03
The balance between privacy, safety and community health. J Paediatr Child Health (2003) 3.00
Linkage relationships of paraoxonase (PON) with other markers: indication of PON-cystic fibrosis synteny. Clin Genet (1985) 2.96
Mapping of mutation causing Friedreich's ataxia to human chromosome 9. Nature (1988) 2.96
Use of penicillin-binding proteins for the identification of enterococci. J Gen Microbiol (1986) 2.87
Screening for carriers of cystic fibrosis through primary health care services. BMJ (1991) 2.84
The positions of three restriction fragment length polymorphisms on chromosome 4 relative to known genetic markers. Hum Genet (1984) 2.84
Microdeletions within 22q11 associated with sporadic and familial DiGeorge syndrome. Genomics (1991) 2.80
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature (1992) 2.76
One or two low affinity penicillin-binding proteins may be responsible for the range of susceptibility of Enterococcus faecium to benzylpenicillin. J Gen Microbiol (1985) 2.73
Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice. Hum Mol Genet (1997) 2.69
Dystrophin-associated proteins are greatly reduced in skeletal muscle from mdx mice. J Cell Biol (1991) 2.68
Molecular basis of muscular dystrophies. Muscle Nerve (2000) 2.65
Overexpression of dystrophin in transgenic mdx mice eliminates dystrophic symptoms without toxicity. Nature (1993) 2.63
Direct binding of G-protein betagamma complex to voltage-dependent calcium channels. Nature (1997) 2.60
Long-term telemetric measurement of cardiovascular parameters in awake mice: a physiological genomics tool. Physiol Genomics (2001) 2.58
Toward a complete linkage map of the human X chromosome: regional assignment of 16 cloned single-copy DNA sequences employing a panel of somatic cell hybrids. Am J Hum Genet (1984) 2.58
Temperature-sensitive mutations in Drosophila melanogaster. VII. A mutation (para-ts) causing reversible adult paralysis. Proc Natl Acad Sci U S A (1971) 2.57
Properties of rapidly labelled deoxyribonucleic acid fragments isolated from the cytoplasm of primary cultures of embryonic mouse liver cells. J Mol Biol (1970) 2.56
Ryanodine receptor of skeletal muscle is a gap junction-type channel. Science (1988) 2.56
Enteroviral protease 2A cleaves dystrophin: evidence of cytoskeletal disruption in an acquired cardiomyopathy. Nat Med (1999) 2.50
The ryanodine receptor/Ca2+ release channel. J Biol Chem (1993) 2.50
Serum matrix metalloproteinase-9 (MMP-9) as a biomarker for monitoring disease progression in Duchenne muscular dystrophy (DMD). Neuromuscul Disord (2011) 2.47
Y chromosome sequence DNA amplified from peripheral blood of women in early pregnancy. Lancet (1994) 2.45
Mutations in the fukutin-related protein gene (FKRP) identify limb girdle muscular dystrophy 2I as a milder allelic variant of congenital muscular dystrophy MDC1C. Hum Mol Genet (2001) 2.45
The use of linked DNA polymorphisms for genotype prediction in families with Duchenne muscular dystrophy. J Med Genet (1983) 2.45
Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice. J Cell Biol (1998) 2.44
Staining of the Ca2+-binding proteins, calsequestrin, calmodulin, troponin C, and S-100, with the cationic carbocyanine dye "Stains-all". J Biol Chem (1983) 2.40
A role for dystroglycan in basement membrane assembly. Cell (1998) 2.36
Dusty starburst galaxies in the early Universe as revealed by gravitational lensing. Nature (2013) 2.35
Isolation of a further anonymous informative DNA sequence from chromosome seven closely linked to cystic fibrosis. Nucleic Acids Res (1986) 2.34
Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy. Lancet (1993) 2.33
Efficient and precise engineering of a 200 kb beta-globin human/bacterial artificial chromosome in E. coli DH10B using an inducible homologous recombination system. Gene Ther (1999) 2.32
Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle. Neuron (1991) 2.30
Unilateral intramuscular injections of acidic saline produce a bilateral, long-lasting hyperalgesia. Muscle Nerve (2001) 2.29
In vivo interaction of beta-lactam antibiotics with the penicillin-binding proteins of Streptococcus pneumoniae. Antimicrob Agents Chemother (1980) 2.29
Congenital muscular dystrophy with merosin deficiency. C R Acad Sci III (1994) 2.22
Report of the DNA committee and catalogues of cloned and mapped genes and DNA polymorphisms. Cytogenet Cell Genet (1990) 2.21
The Ca2+-release channel/ryanodine receptor is localized in junctional and corbular sarcoplasmic reticulum in cardiac muscle. J Cell Biol (1993) 2.18
Chromosome mediated gene transfer of six DNA markers linked to the cystic fibrosis locus on human chromosome seven. Nucleic Acids Res (1986) 2.16
Linkage of COL1A2 collagen gene to cystic fibrosis, and its clinical implications. Lancet (1985) 2.14
Comparison of dose-response curves for alpha factor-induced cell division arrest, agglutination, and projection formation of yeast cells. Implication for the mechanism of alpha factor action. J Biol Chem (1983) 2.13
Muscular dystrophies and the dystrophin-glycoprotein complex. Curr Opin Neurol (1997) 2.13
Prenatal diagnosis of cystic fibrosis by DNA amplification for detection of KM-19 polymorphism. Lancet (1988) 2.13
Delta-beta-thalassemia is due to a gene deletion. Cell (1976) 2.12
Studies on the mechanism of intrinsic resistance to beta-lactam antibiotics in group D streptococci. J Gen Microbiol (1983) 2.10
Dystrophin-glycoprotein complex is highly enriched in isolated skeletal muscle sarcolemma. J Cell Biol (1991) 2.10
Expression of transforming growth factor-beta 1 in dystrophic patient muscles correlates with fibrosis. Pathogenetic role of a fibrogenic cytokine. J Clin Invest (1995) 2.06
The brain ryanodine receptor: a caffeine-sensitive calcium release channel. Neuron (1991) 2.05
Isolation, characterization, and localization of the inositol 1,4,5-trisphosphate receptor protein in Xenopus laevis oocytes. J Biol Chem (1992) 2.05