Pro‐inflammatory immunity supports fibrosis advancement in epidermolysis bullosa: intervention with Ang‐(1‐7)

Autor: Rocco Bernasconi, Kerstin Thriene, Elena Romero‐Fernández, Christine Gretzmeier, Tobias Kühl, Mareike Maler, Pauline Nauroy, Svenja Kleiser, Anne‐Catherine Rühl‐Muth, Michael Stumpe, Dimitra Kiritsi, Stefan F Martin, Boris Hinz, Leena Bruckner‐Tuderman, Jörn Dengjel, Alexander Nyström

Publikováno v: EMBO Molecular Medicine, Vol 13, Iss 10, Pp 1-20 (2021)

Abstract Recessive dystrophic epidermolysis bullosa (RDEB), a genetic skin blistering disease, is a paradigmatic condition of tissue fragility‐driven multi‐organ fibrosis. Here, longitudinal analyses of the tissue proteome through the course of n

Externí odkaz: https://doaj.org/article/58ca17e552d24643b054c0c4e1209d7e

Forschen für die Praxis: Die Rolle epidermaler Proteasen in der Progression der rezessiven dystrophen Epidermolysis bullosa

Autor: Pauline Nauroy, Eleni Zingkou, Dimitra Kiritsi, Georgia Sotiropoulou

Publikováno v: JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 19:828-832

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::e1269e59f1b21798cc89f842593cb60e
https://doi.org/10.1111/ddg.14396_g

The myotendinous junction marker collagen XXII enables zebrafish postural control learning and optimal swimming performance through its force transmission activity

Autor: Sohm F, Allard B, Alexandre Guiraud, Lefrancois C, Malbouyres M, Bernard L, Pauline Nauroy, Salamito M, Florence Ruggiero

Although the myotendinous junction (MTJ) is essential for skeletal muscle integrity, its contribution to skeletal muscle function remains largely unknown. Here, we show that CRISPR-Cas9-mediated gene ablation of the MTJ marker col22a1 in zebrafish id

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::50e737dd8c9442733fb65dd1786b8274
https://doi.org/10.1101/2021.07.14.452354

QR-313, an Antisense Oligonucleotide, Shows Therapeutic Efficacy for Treatment of Dominant and Recessive Dystrophic Epidermolysis Bullosa: A Preclinical Study

Autor: Verena Wally, Douglas R. Keene, M. Peter Marinkovich, Sara F. Tufa, Eva M. Murauer, Elisabeth M. Haisma, Alexander Nyström, Ulrich Koller, Dimitra Kiritsi, Pauline Nauroy, Marieke Hogervorst, Olivier Bornert, Stefan Hainzl, Gerard Platenburg, Ingrid Hausser, Tita Ritsema, Ioannis Athanasiou, Jim Swildens, Johannes Bischof

Publikováno v: The Journal of investigative dermatology. 141(4)

Dystrophic epidermolysis bullosa (DEB) is a blistering skin disease caused by mutations in the gene COL7A1 encoding collagen VII. DEB can be inherited as recessive DEB (RDEB) or dominant DEB (DDEB) and is associated with a high wound burden. Perpetua

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dbf86473d755768081844e5cb869d787
https://pubmed.ncbi.nlm.nih.gov/32946877

Fishing for collagen function: About development, regeneration and disease

Autor: Marilyne Malbouyres, Florence Ruggiero, Sandrine Bretaud, Pauline Nauroy

Publikováno v: Seminars in Cell and Developmental Biology
Seminars in Cell and Developmental Biology, Elsevier, 2019, 89, pp.100-108. ⟨10.1016/j.semcdb.2018.10.002⟩
Seminars in Cell and Developmental Biology, 2019, 89, pp.100-108. ⟨10.1016/j.semcdb.2018.10.002⟩

Collagens are the most abundant vertebrate extracellular matrix proteins. They form a superfamily of 28 members that show a remarkable diversity in molecular and supramolecular organization, tissue distribution and function and mutations in collagen

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::113c7a69da66dc20209a585f57eece9d
https://hal.archives-ouvertes.fr/hal-03484563/file/S1084952117305852.pdf