Results 111 to 120 of about 5,143 (201)

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

A family of membrane-shaping proteins at ER subdomains regulates pre-peroxisomal vesicle biogenesis [PDF]

, 2016
Saccharomyces cerevisiae contains three conserved reticulon and reticulon-like proteins that help maintain ER structure by stabilizing high membrane curvature in ER tubules and the edges of ER sheets.
Agrawal, Agrawal, Agrawal, Amit S. Joshi, Anwar, Bartlett, Bellu, Campbell, Chen, Chen, Choudhary, David, Dawson, Dimitrov, English, Goyal, Haan, Hoepfner, Hu, Hu, Junjie Hu, Khaminets, Kim, Knoops, Lam, Longtine, Mast, Mochida, Motley, Rucktäschel, Santos-Rosa, Shemesh, Shibata, Shibata, Shindiapina, Tim P. Levine, Tolley, Ueda, van der Zand, Van Veldhoven, Vedrenne, Vineet Choudhary, Vizeacoumar, Vizeacoumar, Voeltz, Voss, Waterman-Storer, West, Westrate, William A. Prinz, Williams, Witkin, Xiaofang Huang, Yan, Yonekawa   +54 more
core   +2 more sources

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 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

The Popeye Domain Containing Genes and Their Function in Striated Muscle

, 2016
The Popeye domain containing (POPDC) genes encode a novel class of cAMP effector proteins, which are abundantly expressed in heart and skeletal muscle. Here we will review their role in striated muscle as deduced from work in cell and animal models and ...
Brand, T, Ferlini, A, French, V, Schindler, RFR, Scotton, C   +4 more
core   +2 more sources

Adaptive plasticity in the mouse mandible [PDF]

, 2014
BACKGROUND: Plasticity, i.e. non-heritable morphological variation, enables organisms to modify the shape of their skeletal tissues in response to varying environmental stimuli.
Anderson, Philip S L, Rayfield, Emily J, Renaud, Sabrina   +2 more
core   +6 more sources

Several dystrophin-glycoprotein complex members are present in crude surface membranes but they are sodium dodecyl sulphate invisible in KCl-washed microsomes from mdx mouse muscle. [PDF]

, 2009
International audienceThe dystrophin-glycoprotein complex (DGC) is a large trans-sarcolemmal complex that provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix.
Cherel, Yan, Daval, Stéphanie, Le Rumeur, Elisabeth, Rocher, Chantal   +3 more
core   +3 more sources

Targeted Myostatin Gene Editing in Multiple Mammalian Species Directed by a Single Pair of TALE Nucleases

Molecular Therapy: Nucleic Acids, 2013
Myostatin (MSTN) is a negative regulator of skeletal muscle mass. Strategies to block myostatin signaling pathway have been extensively pursued to increase muscle mass in various disease settings including muscular dystrophy.
Li Xu, Piming Zhao, Andrew Mariano, Renzhi Han   +3 more
doaj   +1 more source

Dysferlin Enables Tubular Membrane Proliferation in Cardiac Hypertrophy

Circulation Research
BACKGROUND: Cardiac hypertrophy compensates for increased biomechanical stress of the heart induced by prevalent cardiovascular pathologies but can result in heart failure if left untreated. Here, we hypothesized that the membrane fusion and repair protein dysferlin is critical for the integrity of the transverse-axial tubule ...
Nora Josefine Paulke, Carolin Fleischhacker, Justus B. Wegener, Gabriel C. Riedemann, Constantin Cretu, Mufassra Mushtaq, Nina Zaremba, Wiebke Möbius, Yannik Zühlke, Jasper Wedemeyer, Lorenz Liebmann, Anastasiia A. Gorshkova, Daniel Kownatzki-Danger, Eva Wagner, Tobias Kohl, Carolin Wichmann, Olaf Jahn, Henning Urlaub, Karl Toischer, Gerd Hasenfuß, Tobias Moser, Julia Preobraschenski, Christof Lenz, Eva A. Rog-Zielinska, Stephan E. Lehnart, Sören Brandenburg   +25 more
openaire   +5 more sources

Osteopontin ablation ameliorates muscular dystrophy by shifting macrophages to a pro-regenerative phenotype. [PDF]

, 2016
In the degenerative disease Duchenne muscular dystrophy, inflammatory cells enter muscles in response to repetitive muscle damage. Immune factors are required for muscle regeneration, but chronic inflammation creates a profibrotic milieu that exacerbates
Barton, Elisabeth R, Capote, Joana, Kramerova, Irina, Martinez, Leonel, Miceli, M Carrie, Spencer, Melissa J, Sweeney, H Lee, Vetrone, Sylvia   +7 more
core   +2 more sources