Results 191 to 200 of about 28,350 (246)

Dystrophin and related proteins

open access: yesCurrent Opinion in Genetics & Development, 1993
During the past year significant progress has been made in understanding how dystrophin deficiency leads to muscle cell necrosis in Duchenne muscular dystrophy and Becker muscular dystrophy. Dystrophin interacts with a glycoprotein complex spanning the muscle sarcolemma, effectively linking the actin cytoskeleton to the extracellular matrix.
J M, Tinsley   +5 more
openaire   +3 more sources

Dystrophin and dystrophin-related protein (utrophin) distribution in normal and dystrophin-deficient skeletal muscles

Neuromuscular Disorders, 1993
The respective localizations of dystrophin and dystrophin-related protein (DRP or utrophin) along the sarcolemmal membrane and at the neuromuscular junctions (NMJs) in normal and dystrophin-deficient skeletal muscles, were determined using confocal laser microscopy. The analysis was prompted by the recent availability of a new anti-utrophin mAb [Bewick
F Pons, L V Nicholson, J J Leger
exaly   +3 more sources

Dystrophin and dystrophin-related proteins: A review of protein and RNA studies

open access: yesNeuromuscular Disorders, 1993
The analysis of dystrophin gene expression has led to the identification of multiple transcripts and varying isoforms. The data indicate that transcription of the dystrophin gene occurs from several promoters, which involves developmental and tissue-dependent regulation.
D R, Love   +4 more
openaire   +3 more sources

Muscle inactivation of mTOR causes metabolic and dystrophin defects leading to severe myopathy [PDF]

open access: yesJournal of Cell Biology, 2009
Mammalian target of rapamycin (mTOR) is a key regulator of cell growth that associates with raptor and rictor to form the mTOR complex 1 (mTORC1) and mTORC2, respectively.
Laetitia Mazelin   +2 more
exaly   +2 more sources

Assembly of the Dystrophin-Associated Protein Complex Does Not Require the Dystrophin Cooh-Terminal Domain [PDF]

open access: yesJournal of Cell Biology, 2000
Dystrophin is a multidomain protein that links the actin cytoskeleton to laminin in the extracellular matrix through the dystrophin associated protein (DAP) complex.
John A Faulkner   +2 more
exaly   +2 more sources

Functions of dystrophin and dystrophin associated proteins

Current Opinion in Neurology, 1997
Dystrophin is a protein product of the X-linked gene mutation that is responsible for Duchenne and Becker muscular dystrophies. The protein binds actin and associates with dystrophin-glycoprotein complex to link the cytoskeleton to the extracellular matrix.
M, Michalak, M, Opas
openaire   +2 more sources

Dystrophin in the retina

Progress in Neurobiology, 1997
Dystrophin is a plasma membrane-associated cytoskeletal protein of the spectrin superfamily. The dystrophin cytoskeleton has been first characterized in muscle. Muscular 427 kDa dystrophin binds to subplasmalemmal actin filaments via its amino-terminal domain.
F, Schmitz, D, Drenckhahn
openaire   +2 more sources

Dystrophin and disease

Molecular Aspects of Medicine, 1991
Recent advances concerning the genetic and biochemical basis of Duchenne and Becker muscular dystrophies have resulted in a good understanding of the etiology of these common dystrophies. An important secondary consequence of the genetic and biochemical research has been the generation of gene-based and protein-based diagnostic tools which enable a ...
E P, Hoffman, L, Schwartz
openaire   +2 more sources

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