Results 221 to 230 of about 7,866 (244)

Active flows drive clustering and sorting of membrane components with differential affinity to dynamic actin cytoskeleton

Bhat A, Das A, Iyer M, Beri S, Talluri S, Koester D, Rao M, Mayor S.   +7 more
europepmc   +1 more source

Actin conformation dynamics precede force generation in cytotoxic T lymphocytes

Lippert A, Rochussen A, Kebabci A, Winkel A, Franze K, Fletcher D, Griffiths G.   +6 more
europepmc   +1 more source

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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.
Jun, Tanihata, Naoki, Suzuki, Yuko, Miyagoe-Suzuki, Kazuhiko, Imaizumi, Shin'ichi, Takeda   +4 more
openaire   +2 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.
J A, Rafael, J M, Tinsley, A C, Potter, A E, Deconinck, K E, Davies   +4 more
openaire   +2 more sources

A study of short utrophin isoforms in mice deficient for full-length utrophin

Mammalian Genome, 2003
Utrophin can functionally replace dystrophin in dystrophin-deficient muscle and may have a role in a therapeutic strategy for Duchenne muscular dystrophy. This has resulted in many investigations of the full-length muscle form of utrophin; however, the short utrophins and non-muscle forms have been relatively neglected, partly because they are ...
Jimenez-Mallebrera C, Davies K, Putt W, Edwards YH   +3 more
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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 ...
C, Rigoletto, A, Prelle, P, Ciscato, M, Moggio, G, Comi, F, Fortunato, G, Scarlato   +6 more
openaire   +2 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.
J M, Tinsley, K E, Davies
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   +3 more sources

Okadaic acid augments utrophin in myogenic cells

Neuroscience Letters, 2004
Duchenne 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, Kyle, Brownback, Michael J, Werle   +2 more
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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 ...
Amy J. Santas, Danielle L. Lavery, Chelsea F. Popowski, April Y. Hung, Ryan E. Hunt, Mary K. Richardson, Kristin M. Braun   +6 more
openaire   +1 more source