Results 231 to 240 of about 7,812 (251)

Downstream utrophin enhancer is required for expression of utrophin in skeletal muscle

The Journal of Gene Medicine, 2008
AbstractBackgroundDuchenne muscular dystrophy is caused by the absence of the muscle cytoskeletal protein dystrophin. Utrophin is an autosomal homologue of dystrophin, and overexpression of utrophin is expected to compensate for the dystrophin deficit.
Kazuhiko Imaizumi, Shin'ichi Takeda, Jun Tanihata, Naoki Suzuki, Yuko Miyagoe-Suzuki   +4 more
openaire   +3 more sources

Utrophin: A potential replacement for dystrophin?

Neuromuscular Disorders, 1993
This 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.
Jonathon M. Tinsley, Kay E. Davies
openaire   +3 more sources

Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice

Nature Genetics, 1998
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease usually resulting in death of patients by their early twenties. In contrast, mice lacking dystrophin (Dmd(mdx)), appear physically normal despite their underlying muscle pathology.
Kay E. Davies, Jonathon M. Tinsley, Anne E. Deconinck, Jill A. Rafael, A Potter   +4 more
openaire   +3 more sources

The NO way to increase muscular utrophin expression?

Comptes Rendus de l'Académie des Sciences - Series III - Sciences de la Vie, 2000
Duchenne muscular dystrophy (DMD), a severe X-linked recessive disorder that results in progressive muscle degeneration, is due to a lack of dystrophin, a membrane cytoskeletal protein. An approach to the search for a treatment is to compensate for dystrophin loss by utrophin, another cytoskeletal protein. During development, in normal as in dystrophic
Chaubourt, Emmanuel, Voisin, Vincent, Fossier, Philippe, Baux, Gérard, Israël, Maurice, de La Porte, Sabine   +5 more
openaire   +4 more sources

Utrophin Expression is Prevalent in Epidermis

BIOS, 2010
Abstract. 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 ...
Kristin M. Braun, Mary K. Richardson, Ryan E. Hunt, April Y. Hung, Amy J. Santas, Danielle L. Lavery, Chelsea F. Popowski   +6 more
openaire   +2 more sources

Utrophin is a calpain substrate in muscle cells

Muscle & Nerve, 2006
AbstractCalpains are Ca2+‐dependent cytosolic cysteine proteases that participate in the pathology of Duchenne muscular dystrophy (DMD). Utrophin is a functional homolog of dystrophin that partially compensates for dystrophin deficiency in myofibers of mdx mice.
Alexandre Briguet, Isabelle Courdier-Fruh   +1 more
openaire   +3 more sources

Utrophin muscles in on the action

Nature Medicine, 1997
Overexpressing utrophin in muscles may prove a promising strategy for treating Duchenne muscular dystrophy but many questions remain unanswered.
openaire   +2 more sources

Muscular nitric oxide synthase (muNOS) and utrophin

Journal of Physiology-Paris, 2002
Duchenne 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, Voisin, Vincent, Fossier, Philippe, Baux, Gérard, Israël, Maurice, de La Porte, Sabine   +5 more
openaire   +4 more sources

Biochemical characterisation of the actin-binding properties of utrophin

Cell Motility and the Cytoskeleton, 2000
Utrophin is a large ubiquitously expressed cytoskeletal protein that is important for maturation of vertebrate neuromuscular junctions. It is highly homologous to dystrophin, the protein defective in Duchenne and Becker muscular dystrophies. Utrophin binds to the actin cytoskeleton via an N-terminal actin-binding domain, which is related to the actin ...
John Kendrick-Jones, Carolyn A. Moores
openaire   +3 more sources

Utrophin expression during human fetal development

International Journal of Developmental Neuroscience, 1995
AbstractUtrophin, 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 ...
Giacomo P. Comi, C. Rigoletto, Guglielmo Scarlato, Patrizia Ciscato, Alessandro Prelle, Maurizio Moggio, F. Fortunato   +6 more
openaire   +3 more sources