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Histopathological assessment of disease target organs in a mouse model of progeria (LMNA G609G/G609G)

Autor: SARLI G., SQUARZONI S., PELLEGRINO V., AVALLONE G., ZAGHINI A., BARBONI C., LOI M., SCHENA E., CAPANNI C., LATTANZI G.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od______4094::4798e1ff682e51fbfc5bb7d6a84cb0b8
http://hdl.handle.net/11585/622482

Effect of mechanical strain on the collagen VI pericellular matrix in anterior cruciate ligament fibroblasts

Autor: Sardone F, Tagliavini F, Pellegrini C, Merlini L, Squarzoni S, Santi S, Neri S, Maraldi N, Sabatelli P., TRAINA, FRANCESCO, FALDINI, CESARE

Publikováno v: Journal of cellular physiology. 229(7)

Cell-extracellular matrix interaction plays a major role in maintaining the structural integrity of connective tissues and sensing changes in the biomechanical environment of cells. Collagen VI is a widely expressed non-fibrillar collagen, which regu

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::3857fb2857bb72b71e00caa5e539a1a0
https://pubmed.ncbi.nlm.nih.gov/24356950

Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis

Autor: Sabatelli P, Gara SK, Grumati P, Zamparelli A, Martoni E, Merlini L, Bonaldo P, Paulsson M, Squarzoni S, Wagener R., GUALANDI, FRANCESCA, PELLEGRINI, CAMILLA, FERLINI, ALESSANDRA, MARALDI, NADIR

Collagen VI is a major extracellular matrix (ECM) protein with a critical role in maintaining skeletal muscle functional integrity. Mutations in COL6A1, COL6A2 and COL6A3 genes cause Ullrich Congenital Muscular Dystrophy (UCMD), Bethlem Myopathy, and

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od______4094::cb169a3393e7b0009f296271f2d1146e
http://hdl.handle.net/11585/121053

Ultrastructural defects of collagen VI filaments in an Ullrich syndrome patient with loss of the alpha3(VI) N10-N7 domains

Autor: Squarzoni S, Sabatelli P, Bergamin N, Guicheney P, Demir E, Merlini L, Lattanzi G, Ognibene A, Capanni C, Mattioli E, Columbaro M, Bonaldo P, MARALDI, NADIR

Publikováno v: Journal of cellular physiology
206:1 (2006): 160–166.
info:cnr-pdr/source/autori:S. SQUARZONI, P. SABATELLI, N. BERGAMIN, P. GUICHENEY, E. DEMIR, L. MERLINI, G. LATTANZI, A. OGNIBENE, C. CAPANNI, E. MATTIOLI, M. COLUMBARO, P. BONALDO, N.M. MARALDI,/titolo:Ultrastructural defects of collagen VI filaments in an Ullrich syndrome patient with loss of the alpha3(VI) N10-N7 domains,/doi:/rivista:Journal of cellular physiology (Print)/anno:2006/pagina_da:160/pagina_a:166/intervallo_pagine:160–166/volume:206:1

Ultrastructural alterations of collagen VI in cultured fibroblasts and reduced collagen VI immunostaining in the papillary dermis and endomysium were detected in a patient with a mild form of Ullrich congenital muscular dystrophy caused by a COL6A3 g

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::48fb842664f988b821c0e707fc2b5006
https://pubmed.ncbi.nlm.nih.gov/15965965