Results 31 to 40 of about 745 (121)
Testing of therapies in a novel nebulin nemaline myopathy model demonstrate a lack of efficacy
Acta Neuropathologica Communications, 2018 Nemaline myopathies are heterogeneous congenital muscle disorders causing skeletal muscle weakness and, in some cases, death soon after birth. Mutations in nebulin, encoding a large sarcomeric protein required for thin filament function, are responsible ...
Tamar E. Sztal +5 more
doaj +1 more source
A Nebulin Ruler Does Not Dictate Thin Filament Lengths [PDF]
, 2009 To generate force, striated muscle requires overlap between uniform-length actin and myosin filaments. The hypothesis that a nebulin ruler mechanism specifies thin filament lengths by targeting where tropomodulin (Tmod) caps the slow-growing, pointed end
Littlefield, Kimberly P. +4 more
core +1 more source
Titin Isoform Size is Not Correlated with Thin Filament Length in Rat Skeletal Muscle
Frontiers in Physiology, 2014 The mechanisms controlling thin filament length in muscle remain controversial. It was recently reported that thin filament length was related to titin size, and that the latter might be involved in thin filament length determination.
Marion Lewis Greaser +1 more
doaj +1 more source
Cytoskeleton, EarlyView.ABSTRACT We here describe mouse models with complementary homozygous Svil mutations. In skeletal muscle, Svil‐Mut mice express the Svil‐encoded N‐terminus fused to the βgal‐neo gene‐trap tag and lack the highly conserved archvillin C‐terminus; Svil‐KO mice lack expression of all known Svil‐encoded proteins; and Svil‐LoxP mice contain loxP sites for ...
Tara C. Smith +9 more
wiley +1 more source
Experimental Physiology, EarlyView.Abstract Ageing is associated with loss of skeletal muscle mass and strength (sarcopenia) and disrupted redox homeostasis. Redox signalling is essential for muscle adaptation, yet the mechanisms by which ageing disrupts cysteine‐based regulation are poorly defined. The drivers of site‐specific reactivity and signalling specificity in aged muscle remain
Ufuk Ersoy, Malcolm J. Jackson
wiley +1 more source
Is nebulin truly a component of the thin filament
, 1997 Nebulin is a protein coextensive with thin filament in skeletal muscle. Several evidences seem to indicate that nebulin may be a component of the thin filament. We prepared thin filaments from rabbit and beef skeletal muscle by extraction of myofibrils
E. Grazi, R. Adami
core +2 more sources
Scientific Reports, 2023 Triple-negative breast cancer (TNBC) subtype is characterized by aggressive clinical behavior and poor prognosis patient outcomes. Here, we show that ADAR1 is more abundantly expressed in infiltrating breast cancer (BC) tumors than in benign tumors ...
Najat Binothman +14 more
doaj +1 more source
Animal Genetics, Volume 57, Issue 5, October 2026.ABSTRACT A previous genome‐wide association study identified regions on canine chromosome (cfa) 13 and 14 associated with early onset myxomatous mitral valve disease (MMVD) in Cavalier King Charles Spaniels (CKCS). In the present study, whole genome sequencing (WGS) of 9 CKCS cases (mitral regurgitation (MR) before 4.5 years or congestive heart failure
Lisbeth Høier Olsen +12 more
wiley +1 more source
Antioxidants, 2023 Nemaline myopathy (NM) is one of the most common forms of congenital myopathy and it is identified by the presence of “nemaline bodies” (rods) in muscle fibers by histopathological examination.
Rocío Piñero-Pérez +12 more
doaj +1 more source
British Journal of Pharmacology, Volume 183, Issue 19, Page 5791-5812, October 2026.Ischaemic stroke is characterised by acute cerebrovascular occlusion and blood–brain barrier (BBB) breakdown. Our results indicated that the histone deacetylase inhibitor suberoylanilide hydroxamic acid (SAHA) ameliorated the loss of BBB integrity, changed the morphology of brain endothelial cells, increased the level of basement membrane and ...
Anikó Szecskó +14 more
wiley +1 more source