Dys- function of dysferlin-deficient hearts. J Mol Med. 2007;85:1203-1214.Wenzel K, Geier C, Qadri F, Hubner N, Schulz H, Erdmann B, Gross V, Bauer D, Dechend R, Dietz R, Osterziel KO, Spuler S, Ozcelik C (2007) Dysfunction of dysferlin-deficient hearts. J Mol Med DOI 10....
Dysferlin-deficient and control mice had different gene expression patterns in terms of cardiomyocyte Z-disc and signal transduction proteins. CapZ, LIM-domain-binding protein 3 (LDB3, MLP), cypher (ZASP), desmin, and the cardiac ankyrin-repeated protein (CARP) were differentially expressed, ...
Interestingly, dysferlin-deficient muscle fibres are defective in Ca2+-dependent sarcolemma resealing. Membrane repair is therefore an active process in skeletal muscle fibres, and dysferlin has an essential role in this process. Our findings show that disruption of the muscle membrane repair machinery...
and a modified glucocorticoid, vamorolone, in experimental models of LGMD2B, vamorolone improved dysferlin-deficient muscle cell membrane stability and repair. This correlated with increasedmuscle strengthand decreasedmuscle degeneration, according to a Children's-led study published online Aug. 27, 201...
Dysferlin-deficient muscular dystrophy is a progressive disease characterized by muscle weakness and wasting for which there is no treatment. It is caused by mutations in DYSF, a large, multiexonic gene that forms a coding sequence of 6.2 kb. Sleeping Beauty (SB) transposon is a nonviral ...
Dysferlin-deficient and control mice had different gene expression patterns in terms of cardiomyocyte Z-disc and signal transduction proteins. CapZ, LIM-domain-binding protein 3 (LDB3, MLP), cypher (ZASP), desmin, and the cardiac ankyrin-repeated protein (CARP) were differentially expressed, ...
Dysferlin-deficient and control mice had different gene expression patterns in terms of cardiomyocyte Z-disc and signal transduction proteins. CapZ, LIM-domain-binding protein 3 (LDB3, MLP), cypher (ZASP), desmin, and the cardiac ankyrin-repeated protein (CARP) were differentially expressed, ...
(2012) GREG cells, a dysferlin-deficient myogenic mouse cell line. Experimental cell research 318, 127-135Humphrey, G.W., Mekhedov, E., Blank, P.S., de Morree, A., Pekkurnaz, G., Nagaraju, K., and Zimmerberg, J. (2012). GREG cells, a dysferlin-deficient myogenic mouse cell ...
Dysfunction of dysferlin- deficient hearts. J Mol Med 2007; 85(11):1203-1214.Wenzel K, Geier C, Qadri F, Hubner N, Schulz H, Erdmann B, Gross V, Bauer D, Dechend R, Dietz R, Osterziel KJ, Spuler S, Ozcelik C. 2007. Dysfunction of dysferlin-deficient hearts. J Mol Med 85(11)...
Interestingly, dysferlin-deficient muscle fibres are defective in Ca[SUP2+]-dependent sarcolemma resealing. Membrane repair is therefore an active process in skeletal muscle fibres, and dysferlin has an essential role in this process. Our findings show that disruption of the muscle membrane repair ...