Results 101 to 110 of about 4,001 (193)

Alpha-tubulin co-localizes with dysferlin.

, 2013
GFP-dysferlin was expressed in C2C12 myoblasts and myotubes. The localization of GFP-dysferlin was compared to that of endogenous alpha-tubulin stained with anti-alpha-tubulin antibody by confocal microscopy.
Bilal A. Azakir (192892), Christian Therrien (192897), Michael Sinnreich (192902), Sabrina Di Fulvio (192887)   +3 more
core   +1 more source

Increased nonHDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B

Journal of Lipid Research, 2018
Progressive limb and girdle muscle atrophy leading to loss of ambulation is a hallmark of dysferlinopathies, which include limb-girdle muscular dystrophy type 2B and Miyoshi myopathy.
Stephanie L. Sellers, Nadia Milad, Zoe White, Chris Pascoe, Rayleigh Chan, Geoffrey W. Payne, Chun Seow, Fabio Rossi, Michael A. Seidman, Pascal Bernatchez   +9 more
doaj   +1 more source

Nanodysferlins support membrane repair and binding to TRIM72/MG53 but do not localize to t-tubules or stabilize Ca2+ signaling

Molecular Therapy: Methods & Clinical Development
Mutations in the DYSF gene, encoding the protein dysferlin, lead to several forms of muscular dystrophy. In healthy skeletal muscle, dysferlin concentrates in the transverse tubules and is involved in repairing the sarcolemma and stabilizing Ca2 ...
Joaquin Muriel, Valeriy Lukyanenko, Thomas A. Kwiatkowski, Yi Li, Sayak Bhattacharya, Kassidy K. Banford, Daniel Garman, Hannah R. Bulgart, Roger B. Sutton, Noah Weisleder, Robert J. Bloch   +10 more
doaj   +1 more source

Dysferlin-deficient muscular dystrophy features amyloidosis

Objective: Dysferlin (DYSF) gene mutations cause limb girdle muscular dystrophy type 2B and Miyoshi\u27s myopathy. The consequences of DYSF mutations on protein structure are poorly understood.
Bushby K, Moore SA, Carl M, Bähring S, Rocken C, Spuler S, Straub V, Wenzel K, Vinkemeier U, Zabojszcza J   +9 more
core   +5 more sources

Dysferlin deficiency alters lipid metabolism and remodels the skeletal muscle lipidome in mice

Journal of Lipid Research, 2019
Defects in the gene coding for dysferlin, a membrane-associated protein, affect many tissues, including skeletal muscles, with a resultant myopathy called dysferlinopathy.
Vanessa R. Haynes, Stacey N. Keenan, Jackie Bayliss, Erin M. Lloyd, Peter J. Meikle, Miranda D. Grounds, Matthew J. Watt   +6 more
doaj   +1 more source

Mapping the determinants of dysferlin dimerization.

, 2013
(A) Dependence of FRET efficiencies on the protein expression levels of C2A to C2D domains of dysferlin. Hyperbolic fitting showed that the C2B (red), C2C (green) and C2D (purple) domains of dysferlin all mediate the self-interaction.
Sandeep Pallikkuth (339527), Zhanjia Hou (316171), Seth L. Robia (316175), Gregory A. Mignery (339528), Renzhi Han (276195), Li Xu (108702)   +5 more
core   +1 more source

Angiotensin II receptor blocker losartan exacerbates muscle damage and exhibits weak blood pressure-lowering activity in a dysferlin-null model of Limb-Girdle muscular dystrophy type 2B.

PLoS ONE, 2019
There is no cure or beneficial management option for Limb-Girdle muscular dystrophy (MD) type 2B (LGMD2B). Losartan, a blood pressure (BP) lowering angiotensin II (AngII) receptor type 1 (ATR1) blocker (ARB) with unique anti-transforming growth factor-β (
Zoe White, Nadia Milad, Arash Y Tehrani, William Wei-Han Chen, Graham Donen, Stephanie L Sellers, Pascal Bernatchez   +6 more
doaj   +1 more source

Dysferlin [PDF]

, 2020
openaire   +1 more source

Immunoblot validation and characterization of dysferlin.

, 2014
A) Western blot of several proteins that were increased or decreased in DBA patients including dysferlin (DYSF), gamma-adducin (ADD3), major vault protein (MVP), serine palmitoyltransferase 1 and 2 (SPTLC1 and SPTLC2), HLA-ABC, and intercellular adhesion
Monica Bessler (508914), Hsin-Yao Tang (395357), David W. Speicher (395361), Sira Sriswasdi (42545), Esther N. Pesciotta (508912), Philip J. Mason (508913)   +5 more
core   +1 more source

Dysferlin (DYSF) [PDF]

Science-Business eXchange, 2014
openaire   +1 more source