<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Vafiadaki, E.</style></author><author><style face="normal" font="default" size="100%">Arvanitis, DA</style></author><author><style face="normal" font="default" size="100%">Papalouka, V.</style></author><author><style face="normal" font="default" size="100%">Terzis, G.</style></author><author><style face="normal" font="default" size="100%">Roumeliotis, T. I.</style></author><author><style face="normal" font="default" size="100%">Spengos, K.</style></author><author><style face="normal" font="default" size="100%">Garbis, S.D.</style></author><author><style face="normal" font="default" size="100%">Manta, P</style></author><author><style face="normal" font="default" size="100%">Kranias, EG</style></author><author><style face="normal" font="default" size="100%">Sanoudou, D.</style></author></authors></contributors><titles><title><style face="normal" font="default" size="100%">Muscle lim protein isoform negatively regulates striated muscle actin dynamics and differentiation</style></title><secondary-title><style face="normal" font="default" size="100%">FEBS JFEBS JFEBS J</style></secondary-title><alt-title><style face="normal" font="default" size="100%">The FEBS journal</style></alt-title><short-title><style face="normal" font="default" size="100%">The FEBS journalThe FEBS journal</style></short-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Actins/*metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Alternative Splicing</style></keyword><keyword><style  face="normal" font="default" size="100%">Amino Acid Sequence</style></keyword><keyword><style  face="normal" font="default" size="100%">Animals</style></keyword><keyword><style  face="normal" font="default" size="100%">Cell Differentiation</style></keyword><keyword><style  face="normal" font="default" size="100%">Cell Line</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">LIM Domain Proteins/blood/chemistry/genetics/*metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Mice</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle Development</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle Proteins/blood/chemistry/genetics/*metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle, Skeletal/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Muscle, Striated/*cytology</style></keyword><keyword><style  face="normal" font="default" size="100%">Myoblasts/physiology</style></keyword><keyword><style  face="normal" font="default" size="100%">Neuromuscular Diseases/physiopathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Protein Isoforms/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Sequence Alignment</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2014</style></year><pub-dates><date><style  face="normal" font="default" size="100%">Jul</style></date></pub-dates></dates><number><style face="normal" font="default" size="100%">14</style></number><volume><style face="normal" font="default" size="100%">281</style></volume><pages><style face="normal" font="default" size="100%">3261-79</style></pages><isbn><style face="normal" font="default" size="100%">1742-4658 (Electronic)1742-464X (Linking)</style></isbn><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">Muscle lim protein (MLP) has emerged as a critical regulator of striated muscle physiology and pathophysiology. Mutations in cysteine and glycine-rich protein 3 (CSRP3), the gene encoding MLP, have been directly associated with human cardiomyopathies, whereas aberrant expression patterns are reported in human cardiac and skeletal muscle diseases. Increasing evidence suggests that MLP has an important role in both myogenic differentiation and myocyte cytoarchitecture, although the full spectrum of its intracellular roles has not been delineated. We report the discovery of an alternative splice variant of MLP, designated as MLP-b, showing distinct expression in neuromuscular disease and direct roles in actin dynamics and muscle differentiation. This novel isoform originates by alternative splicing of exons 3 and 4. At the protein level, it contains the N-terminus first half LIM domain of MLP and a unique sequence of 22 amino acids. Physiologically, it is expressed during early differentiation, whereas its overexpression reduces C2C12 differentiation and myotube formation. This may be mediated through its inhibition of MLP/cofilin-2-mediated F-actin dynamics. In differentiated striated muscles, MLP-b localizes to the sarcomeres and binds directly to Z-disc components, including alpha-actinin, T-cap and MLP. The findings of the present study unveil a novel player in muscle physiology and pathophysiology that is implicated in myogenesis as a negative regulator of myotube formation, as well as in differentiated striated muscles as a contributor to sarcomeric integrity.</style></abstract><accession-num><style face="normal" font="default" size="100%">24860983</style></accession-num><notes><style face="normal" font="default" size="100%">Vafiadaki, ElizabethArvanitis, Demetrios APapalouka, VasilikiTerzis, GerasimosRoumeliotis, Theodoros ISpengos, KonstantinosGarbis, Spiros DManta, PanagiotaKranias, Evangelia GSanoudou, DespinaengR01 HL026057/HL/NHLBI NIH HHS/R01 HL064018/HL/NHLBI NIH HHS/Research Support, Non-U.S. Gov'tEngland2014/05/28 06:00FEBS J. 2014 Jul;281(14):3261-79. doi: 10.1111/febs.12859. Epub 2014 Jun 11.</style></notes><custom2><style face="normal" font="default" size="100%">4416226</style></custom2><auth-address><style face="normal" font="default" size="100%">Molecular Biology Division, Biomedical Research Foundation of the Academy of Athens, Greece.</style></auth-address></record></records></xml>