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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   +6 more
openaire   +1 more source

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   +6 more
openaire   +2 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 ...
C A, Moores, J, Kendrick-Jones
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   +5 more
openaire   +3 more sources

Expression and localization of utrophin in differentiating PC12 cells

NeuroReport, 2000
To ascertain the role of utrophin in cultured neuronal cells, we investigated its expression and distribution along the NGF-induced differentiation of PC12 cells grown on different substrata. Utrophin mRNA was measured by RT-PCR assay and utrophin protein was quantified by immunoblot analysis.
H, Rosas-Vargas   +6 more
openaire   +2 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   +2 more
openaire   +2 more sources

[The regulation of UTROPHIN expression by EN1].

Yi chuan = Hereditas, 2011
To investigate possible factors up-regulating the expression of UTROPHIN, potential regulatory elements in the promoter of the human UTROPHIN was predicted by P-match software and verified by EMSA and ChIP. The mechanism of EN1 regulation of the human UTROPHIN expression was evaluated by RNA interference and real-time PCR analyses.
Qian, Wang   +5 more
openaire   +1 more source

Utrophin to the rescue

Nature, 1996
Kevin P. Campbell, Rachelle H. Crosbie
openaire   +1 more source

Disruption of the utrophin-actin interaction by monoclonal antibodies and prediction of an actin-binding surface of utrophin.

The Biochemical journal, 1999
Monoclonal antibody (mAb) binding sites in the N-terminal actin-binding domain of utrophin have been identified using phage-displayed peptide libraries, and the mAbs have been used to probe functional regions of utrophin involved in actin binding. mAbs were characterized for their ability to interact with the utrophin actin-binding domain and to affect
G E, Morris   +5 more
openaire   +1 more source

Utrophin

Journal of the Neurological Sciences, 2002
openaire   +2 more sources

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