Online citations, reference lists, and bibliographies.
← Back to Search

Duchenne Muscular Dystrophy: Deficiency Of Dystrophin At The Muscle Cell Surface

E. Bonilla, C. Samitt, A. Miranda, A. Hays, L. Rowland
Published 1988 · Biology, Medicine

Cite This
Download PDF
Analyze on Scholarcy
Share
Dystrophin is the altered gene product in Duchenne muscular dystrophy (DMD). We used polyclonal antibodies against dystrophin to immunohistochemically localize the protein in human muscle. In normal individuals and in patients with myopathies other than DMD, dystrophin was localized to the sarcolemma of the fibers. The protein was absent or markedly deficient in DMD. The sarcolemmal localization of dystrophin is consistent with other evidence that there are structural and functional abnormalities of muscle surface membranes in DMD.
This paper references
10.1212/WNL.33.10.1368
Muscle plasma membrane abnormalities in infants with Duchenne muscular dystrophy
Y. Wakayama (1983)
10.1002/ANA.410220420
Genetic heterogeneity in Duchenne Dystrophy
Craig Hyser (1987)
10.1126/SCIENCE.3659917
Conservation of the Duchenne muscular dystrophy gene in mice and humans.
E. Hoffman (1987)
10.1056/NEJM198805263182104
Characterization of dystrophin in muscle-biopsy specimens from patients with Duchenne's or Becker's muscular dystrophy.
E. Hoffman (1988)
Freeze-fracture studies of muscle caveolae in human muscular dystrophy.
E. Bonilla (1981)
10.1212/WNL.25.12.1111
Duchenne dystrophy
B. Mokri (1975)
10.1001/ARCHNEUR.1960.03840090124020
Handbook of Physiology.
F. Plum (1960)
10.1016/0092-8674(88)90503-X
α-actinins and the DMD protein contain spectrin-like repeats
M. Davison (1988)
10.1016/0022-510X(84)90114-X
Altered binding of Ricinus communis I lectin by muscle membranes in Duchenne muscular dystrophy
M. J. Capaldi (1984)
10.1001/ARCHNEUR.1974.00490310098016
Adenyl cyclase in normal and pathologic human muscle.
S. Mawatari (1974)
10.1083/JCB.47.2.488
STUDIES OF THE TRIAD I. Structure of the Junction in Frog Twitch Fibers
C. Franzini-armstrong (1970)
10.1016/0092-8674(87)90579-4
Dystrophin: The protein product of the duchenne muscular dystrophy locus
E. Hoffman (1987)
10.1016/0092-8674(87)90504-6
Complete cloning of the duchenne muscular dystrophy (DMD) cDNA and preliminary genomic organization of the DMD gene in normal and affected individuals
M. Koenig (1987)
10.1038/328434A0
A cDNA clone from the Duchenne/Becker muscular dystrophy gene
A. Burghes (1987)
10.1146/ANNUREV.BI.54.070185.001421
The membrane skeleton of human erythrocytes and its implications for more complex cells.
V. Bennett (1985)
10.1002/MUS.880030103
Biochemistry of muscle membranes in Duchenne muscular dystrophy
L. Rowland (1980)
Muscle biopsy: A modern approach
V. Dubowitz (1973)
10.1016/0092-8674(87)90631-3
From genes to structural morphogenesis: The genesis and epigenesis of a red blood cell
E. Lazarides (1987)
10.1038/323646A0
Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy gene
A. Monaco (1986)
Progressive muscular dystrophy
Pinheiro Gc (1959)
10.1212/WNL.31.3.331
(Na+ + K+) Mg2+‐ATPase of muscle plasma membranes in Duchenne muscular dystrophy
I. Niebrój-Dobosz (1981)
10.1172/JCI102950
Biochemical study of muscle in progressive muscular dystrophy.
J. Dreyfus (1954)
10.1083/JCB.38.1.1
NONDROPLET ULTRASTRUCTURAL DEMONSTRATION OF CYTOCHROME OXIDASE ACTIVITY WITH A POLYMERIZING OSMIOPHILIC REAGENT, DIAMINOBENZIDINE (DAB)
A. Seligman (1968)
10.1093/BRAIN/102.1.147
Duchenne muscular dystrophy: plasma membrane loss initiates muscle cell necrosis unless it is repaired.
S. Carpenter (1979)
10.1002/ANA.410040205
Duchenne dystrophy: Focal alterations in the distribution of concanavalin a binding sites at the muscle cell surface
E. Bonilla (1978)
10.1038/330754A0
Subcellular fractionation of dystrophin to the triads of skeletal muscle
E. Hoffman (1987)
10.1002/MUS.880030104
Application of the freeze fracture technique to the study of human neuromuscular disease
Donald L. Schotland (1980)



This paper is referenced by
10.1083/JCB.108.2.503
Detection of a specific isoform of alpha-actinin with antisera directed against dystrophin
E. Hoffman (1989)
10.1007/BF01974576
Increased susceptibility to lipid peroxidation in skeletal muscles of dystrophic hamsters
A. Salminen (2005)
10.1016/0968-0004(89)90290-9
Dystrophin, the protein product of the Duchenne/Becker muscular dystrophy gene.
A. Monaco (1989)
10.1016/0887-8994(90)90071-8
Dystrophin: a clinical perspective.
H. Wessel (1990)
10.1016/0009-8981(95)06257-2
Effect of prednisone on protease activities and structural protein levels in rat muscles in vivo.
J. Haycock (1996)
Inflammation and Stem Cell Transplantation
K. Urish (2007)
10.3181/0804-RM-134
Regulation of Cell Proliferation by Fast Myosin Light Chain 1 in Myoblasts Derived from Extraocular Muscle, Diaphragm and Gastrocnemius
S. Zhang (2008)
10.1387/IJDB.1295566
Patterns of dystrophin expression in developing, adult and regenerating tail skeletal muscle of Amphibian urodeles.
J. Arsanto (1992)
10.1007/BF00208944
A normal male with an inherited deletion of one exon within the DMD gene
M. Nordenskjöld (2004)
Mechanical Regulation of Striated Muscle Nitric Oxide Signaling in Muscular Dystrophy
Joanne F. Garbincius (2017)
10.18535/JMSCR/V7I6.154
A Study on Duchenne Muscular Dystrophy (DMD)
Rajendra Kumar Choudhary (2019)
10.1212/WNL.40.5.808
Enormous dystrophin in a patient with Becker muscular dystrophy
C. Angelini (1990)
10.1212/WNL.48.6.1633
DMD‐specific FISH probes are diagnostically useful in the detection of female carriers of DMD gene deletions
A. Voskova-Goldman (1997)
10.1093/qjmed/hcx220
Cardiomyopathy in muscular dystrophy.
R. Indorkar (2018)
10.1016/j.ijcard.2020.05.052
Duchenne and Becker muscular dystrophy carriers: Evidence of cardiomyopathy by exercise and cardiac MRI testing.
M. Mah (2020)
10.1101/2020.04.17.017822
Transplantation of Muscle Stem Cell Mitochondria Rejuvenates the Bioenergetic Function of Dystrophic Muscle
M. Mohiuddin (2020)
10.1016/0968-4328(93)90055-6
Ultrastructure of muscular dystrophy: New aspects
M. Cullen (1993)
10.1016/0960-8966(93)90003-3
Dystrophin-positive myotubes in innervated muscle cultures from Duchenne and Becker muscular dystrophy patients
M. Fanin (1993)
10.1136/adc.68.5.632
Functional significance of dystrophin positive fibres in Duchenne muscular dystrophy.
L. Nicholson (1993)
10.1016/0960-8966(94)90018-3
Expression of NCAM and its polysialylated isoforms during mdx mouse muscle regeneration and in vitro myogenesis
C. Dubois (1994)
10.1089/HUM.1997.8.4-439
LacZ gene transfer to skeletal muscle using a replication-defective herpes simplex virus type 1 mutant vector.
J. Huard (1997)
10.1023/A:1009805419285
Dystrophin and the Cardiomyocyte Membrane Cytoskeleton in the Healthy and Failing Heart
R. Kaprielian (2004)
10.1111/febs.12273
Recent progress in satellite cell/myoblast engraftment – relevance for therapy
D. Briggs (2013)
10.46582/jsrm.0702013
Direct myocardial implantation of human embryonic stem cells in a dog model of Duchenne cardiomyopathy reveals poor cell survival in dystrophic tissue
Walter Habeler (2011)
10.1016/0306-4522(92)90288-D
Localization of dystrophin and dystrophin-related protein at the electromotor synapse and neuromuscular junction in Torpedo marmorata
A. Cartaud (1992)
10.1212/WNL.39.8.1011
Improved diagnosis of Becker muscular dystrophy by dystrophin testing
E. Hoffman (1989)
Molecular mechanisms in muscular dystrophy : a gene expression profiling study.
Rolf Turk (2006)
10.1212/WNL.43.4.795
Duchenne muscular dystrophy
K. Ohlendieck (1993)
10.1016/0022-510X(90)90263-M
Dystrophin immunostaining in muscles from patients with different types of muscular dystrophy: a Brazilian study
M. Vainzof (1990)
10.1517/14728214.2012.691965
Emerging drugs for Duchenne muscular dystrophy
Vinod Malik (2012)
10.1111/jnc.13269
Treatment with human immunoglobulin G improves the early disease course in a mouse model of Duchenne muscular dystrophy
J. Zschüntzsch (2016)
An investigation of the stem cell potential of skeletal muscle satellite cells.
C. Collins (2004)
See more
Semantic Scholar Logo Some data provided by SemanticScholar