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Utrophin expression during human fetal development
International Journal of Developmental Neuroscience, 1995AbstractUtrophin, a protein encoded by chromosome 6 is highly homologous to the cysteine‐rich domain and most of the C‐terminal domain of dystrophin. In order to clarify its functional role we analyzed its expression during human fetal development. We carried out immunohistochemical analysis on muscle from normal human fetuses at different ages of ...
C, Rigoletto +6 more
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Utrophin: A potential replacement for dystrophin?
Neuromuscular Disorders, 1993This paper reviews the evidence that utrophin, the autosomally encoded protein related to dystrophin, may be capable of performing the same cellular functions as dystrophin. If this is the case, it may be possible to modify the regulation of utrophin expression as an alternative route to dystrophin gene therapy for sufferers of DMD and/or BMD.
J M, Tinsley, K E, Davies
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Muscular nitric oxide synthase (muNOS) and utrophin
Journal of Physiology-Paris, 2002Duchenne muscular dystrophy (DMD), the severe X-linked recessive disorder which results in progressive muscle degeneration, is due to a lack of dystrophin, a membrane cytoskeletal protein. Three types of treatment are envisaged: pharmacological (glucocorticoid), myoblast transplantation, and gene therapy.
Chaubourt, Emmanuel +5 more
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Okadaic acid augments utrophin in myogenic cells
Neuroscience Letters, 2004Duchenne muscular dystrophy is a fatal childhood disease caused by mutations that abolish the expression of dystrophin in muscle. Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle. A potential therapeutic approach is to increase utrophin levels in muscle.
Marianna, Rodova +2 more
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Dystrophin, Utrophin, and Muscular Dystrophy
New England Journal of Medicine, 1995exaly +2 more sources
Utrophin Expression is Prevalent in Epidermis
BIOS, 2010Abstract. Utrophin protein was discovered to localize to the neuromuscular junctions and is thought to provide a critical linkage between integral proteins bound to the extracellular matrix outside the cell and the actin cytoskeleton inside the cell. Clinical implications of this protein include the upregulation of utrophin as a treatment for Duchenne ...
Amy J. Santas +6 more
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Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene [PDF]
Gene Therapy, 2000 Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disorder caused by the lack of a subsarcolemmal protein, dystrophin. We have previously shown that the dystrophin-related protein, utrophin is able to compensate for the lack of dystrophin in the mdx mouse, the mouse model for DMD.
Wakefield, P. M. +5 more
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Utrophin muscles in on the action
Nature Medicine, 1997Overexpressing utrophin in muscles may prove a promising strategy for treating Duchenne muscular dystrophy but many questions remain unanswered.
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