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The effect of dietary crude protein (CP) reduction, supplementation with arginine or leucine on intramuscular fat (IMF) content was evaluated in (Landrace × Duroc) × Pietrain pigs. One-hundred and eight barrows (67 ± 4 kg) were assigned to six diets (n=6 pens of 3 pigs each): four normal CP diets containing 16% CP from 60 to 90 kg and 13% CP from 90 to 115 kg live weight (normal protein; normal protein high Arg, normal protein high Leu or normal protein high Arg and Leu) and two low CP diets containing 14% CP from 60 to 90 kg and 11.8% CP from 90 to 115 kg live weight (with or without supplementation of both amino acids). The high Leu and Arg diets were supplemented to obtain ratios of standard ileal digestible Leu/Lys and Arg/Lys of 4 and 2, respectively. While feed to gain ratio tended to increase (p

Laminopathies are causally associated with mutations on the Lamin A/C gene (LMNA). To date, more than 400 mutations in LMNA have been reported in patients. These mutations are widely distributed throughout the entire gene and are associated with a wide range of phenotypes. Unfortunately, little is known about the mechanisms underlying the effect of the majority of these mutations. This is the case of more than 40 mutations that are located at exon 4. Using CRISPR/Cas9 technology, we generated a collection of Lmna exon 4 mutants in mouse C2C12 myoblasts. These cell models included different types of exon 4 deletions and the presence of R249W mutation, one of the human variants associated with a severe type of laminopathy, LMNA-associated congenital muscular dystrophy (L-CMD). We characterized these clones by measuring their nuclear circularity, myogenic differentiation capacity in 2D and 3D conditions, DNA damage, and levels of p-ERK and p-AKT (phosphorylated Mitogen-Activated Protein Kinase 1/3 and AKT serine/threonine kinase 1). Our results indicated that Lmna exon 4 mutants showed abnormal nuclear morphology. In addition, levels and/or subcellular localization of different members of the lamin and LINC (LInker of Nucleoskeleton and Cytoskeleton) complex were altered in all these mutants. Whereas no significant differences were observed for ERK and AKT activities, the accumulation of DNA damage was associated to the Lmna p.R249W mutant myoblasts. Finally, significant myogenic differentiation defects were detected in the Lmna exon 4 mutants. These results have key implications in the development of future therapeutic strategies for the treatment of laminopathies.

Los estudios más recientes de la historia cultural de la política en su vertiente dedicada al estudio de las representaciones nacionales han prestado gran atención a la estatuaria monumental de naturaleza política o nacional. En el mundo hispano –tanto en España, como en el resto de repúblicas hispanoamericanas– existe un parque monumental de tintes nacionales que contribuyó a la construcción nacional y a la nacionalización más o menos exitosa de sus ciudadanos. Situados en el período de apogeo de la estatuaria monumental de carácter nacional, entre los años finiseculares decimonónicos y los años novoseculares del novecientos, aparece la figura de Miquel Blay (Olot, 1866 – Madrid, 1936), prolífico escultor catalán que, a diferencia de lo que han proclamado los sucesivos estudios monográficos sobre el autor, dedicó gran parte de su producción a la estatuaria monumental. Empezando con la representación escultórica de un mito nacional procedente de su región de origen –el de los sitios de Gerona en 1808 y 1809–, Blay continuó por esta senda con la preparación de numerosas obras monumentales que encarnaban, ya sea los valores patrios, ya sea los héroes y los mitos nacionales justificativos de la existencia de una nación contemporánea. A la llegada de su muerte en 1936 la abundante obra de Blay, realizada o no realizada, era mayoritariamente monumental y de carácter nacional, nacionalista o con fines nacionalizadores.