Results 81 to 90 of about 745 (121)

A six-module human nebulin fragment bundles actin filaments and induces actin polymerization

, 1998
Gonsior SM, Gautel M, Hinssen H. A six-module human nebulin fragment bundles actin filaments and induces actin polymerization. JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY. 1998;19(3):225-235.We have investigated the interaction of a 6-repeat recombinant
Gautel, M, Hinssen, Horst, Gonsior, SM
core   +1 more source

Generation of a biallelic NRAP-knockout mutant from a human iPSC line

Stem Cell Research
Cardiomyopathies, a leading cause of mortality, are associated with dysfunctional intercalated discs, which connect neighbouring cardiomyocytes and ensure proper contractility.
Janice Raabe, Vanessa Lewandowski, Sigrid Fuchs, Alexandra Hammerschmidt, Angelika Piasecki, Ellen Orthey, Elisabeth Krämer, Elisabeth Ehler, Friederike Cuello   +8 more
doaj   +1 more source

Influence of nebulin on the assembly mechanics of desmin [PDF]

, 2018
Desmin intermediate filaments (DIFs) bind to nebulin, forming a direct link between the intermediate filament (IF) cytoskeletal network and the Z-discs of myofibrils in muscle cells.
Caragea, Marc Adrian
core   +2 more sources

Generation of a novel mouse model of nemaline myopathy due to recurrent NEB exon 55 deletion

Skeletal Muscle
Biallelic pathogenic variants in the nebulin (NEB) gene lead to the congenital muscle disease nemaline myopathy. In-frame deletion of exon 55 (ΔExon55) is the most common disease-causing variant in NEB.
Zachary Coulson, Justin Kolb, Nesrin Sabha, Esmat Karimi, Zaynab Hourani, Coen Ottenheijm, Henk Granzier, James J. Dowling   +7 more
doaj   +1 more source

Proteômica e sepse: novas perspectivas para o diagnóstico Proteomics and sepsis: new perspectives for diagnosis

Revista Brasileira de Terapia Intensiva, 2007
JUSTIFICATIVA E OBJETIVOS: O diagnóstico e o tratamento da sepse continuam a desafiar a todos; e desenvolver formas mais precisas de abordagem são absolutamente necessárias.
Afonso J. C. Soares, Marise F. Santos, Janete Chung, Cid Marcos N David, Gilberto B. Domont   +4 more
doaj   +1 more source

A peptidomic approach to discover the potential biomolecular signatures discriminating in-bag dry- and wet-aged lamb

Food Chemistry Advances
The study assessed the peptidomic profile of in-bag dry-aged (2 °C, RH 75 %, 0.5 m.s−1, n = 30) and wet-aged (-1.5 °C, n = 30) lamb with high and low levels of lipid oxidation.
Renyu Zhang, Evelyne Maes, Charles Hefer, Alasdair Noble, Ancy Thomas, Michelle J.Y. Yoo, Mustafa M. Farouk, Carolina E. Realini, Santanu Deb-Choudhury   +8 more
doaj   +1 more source

Generation of a NRAP-overexpressing mutant from a human iPSC line

Stem Cell Research
Cardiomyopathies are a major contributor to cardiovascular mortality and are frequently linked to abnormalities in intercalated discs, which coordinate mechanical and electrical signaling between cardiomyocytes.
Janice Raabe, Sigrid Fuchs, Christa Augustin, Alexandra Hammerschmidt, Elisabeth Krämer, Ellen Orthey, Elisabeth Ehler, Friederike Cuello   +7 more
doaj   +1 more source

In vivo and in vitro investigations of heterozygous nebulin knock-out mice disclose a mild skeletal muscle phenotype

, 2013
Nemaline myopathy is the most common congenital skeletal muscle disease, and mutations in the nebulin gene account for 50% of all cases. Recent studies suggest that the disease severity might be related to the nebulin expression levels.
Gretz, N., Jubeau, M., Vilmen, C., Granzier, H., Witt, C.C., Gineste, C., Le Fur, Y., Kohl, C., Stienen, G.J.M., Brohm, K., Gondin, J., de Winter, J.M., Pecchi, E., Bendahan, D., Labeit, S., Ottenheijm, C.A.C., Giannesini, B., Cozzone, P.J.   +17 more
core   +1 more source

Sarcoplasmic reticulum calcium uptake and speed of relaxation are depressed in nebulin-free skeletal muscle

, 2008
Previous work suggested that altered Ca2+ homeostasis might contribute to dysfunction of nebulinfree muscle, as gene expression analysis revealed that the sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA)-inhibitor sarcolipin (SLN) is up-regulated >70 ...
Vangheluwe, Peter, Wuytack, Frank, Babu, Gopal J., Ottenheijm, Coen A.C., Witt, Christian C., Fong, Chi, Granzier, Henk, Periasamy, Muthu, Labeit, Siegfried   +8 more
core   +1 more source

Serum Proteomics Provides Novel Biomarkers of Inflammation, Tissue Injury, and Therapeutic Response in Experimental Chagas Disease

Microorganisms
Chagas disease remains a major public health challenge and still lacks reliable serum biomarkers capable of accurately reflecting disease progression and therapeutic response.
Eloan Mendes Vieira, Camilo Elber Vital, Paula Melo de Abreu Vieira, Lorena Cera Bandeira, Luciana da Fonseca Medeiros, Nívia Carolina Nogueira Paiva, William de Castro Borges, Cláudia Martins Carneiro   +7 more
doaj   +1 more source