Conhecimento e natureza na filosofía da David Hum

Autor: José Claudio Morelli

Publikováno v: Enfoques, Vol 21, Iss 1-2 (2016)

La filosofía de Hume fue, durante mucho tiempo, percibida como una filosofía escéptica. Una interpretación naturalista pretende, entre tanto, atribuir nuevos sentidos al aspecto afirmativo de su filosofía, mostrando que Hume estaba interesado en

Externí odkaz: https://doaj.org/article/3bd663abb9e2471a9c6b77fc55cf84e6

Biochemically deleterious human NFKB1 variants underlie an autosomal dominant form of common variable immunodeficiency

Autor: Peng Zhang, Katharina Thoma, Bingnan Lyu, Gulbu Uzel, Juan Li, Boualem Hammadi, András N Spaan, Jamila El Baghdadi, Alexandra F. Freeman, Charlotte Cunningham-Rundles, Yu Zhang, Jean-Laurent Casanova, Claire Fieschi, Franck Rapaport, Jérémie Rosain, Vivien Béziat, Simon J. Pelham, Wei-Te Lei, Anne Puel, Maya Chrabieh, Helen C. Su, V. Koneti Rao, David Hum, Stéphanie Boisson-Dupuis, Jacinta Bustamante, Mélanie Migaud, Anne-Sophie Korganow, Qian Zhang, Aurélie Cobat, Steven M. Holland, Laurent Abel, Vishukumar Aimanianda, Bertrand Boisson, Manfred Fliegauf, Benedetta Bigio, Bodo Grimbacher, Yoann Seeleuthner, Takaki Asano, Carol J Saunders, Shen-Ying Zhang, Emmanuelle Jouanguy

Publikováno v: Journal of Experimental Medicine. 218

Autosomal dominant (AD) NFKB1 deficiency is thought to be the most common genetic etiology of common variable immunodeficiency (CVID). However, the causal link between NFKB1 variants and CVID has not been demonstrated experimentally and genetically,

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d5ac8d2da4117487a80552a370fb8fde
https://doi.org/10.1084/jem.20210566

The human CIB1–EVER1–EVER2 complex governs keratinocyte-intrinsic immunity to β-papillomaviruses

Autor: Matthieu Bouaziz, Sirous Zeinali, Vignesh Gunasekharan, Fabienne Jabot-Hanin, Sarah Jill De Jong, Peter Itin, Nessa Aghazadeh, Xavier Rueda Cadena, E. Imahorn, Aurelia D’Amico, Amandine Crequer, Irina Matos, Elaine Fuchs, Bettina Burger, Janet Markle, David Hum, Etienne Patin, Florian Full, Bayaki Saka, Amir Hossein Saeidian, Laurent Abel, James G. Krueger, Nataly Portilla Maya, Claire Q.F. Wang, Jouni Uitto, Emmanuelle Jouanguy, Leila Youssefian, Hassan Vahidnezhad, Armin Ensser, Andrés Augusto Arias, Leslie V. Parise, Tina M. Leisner, Jean-Laurent Casanova, Lou Laimins, Lazaro Lorenzo, Gérard Orth, José Luis Franco

Publikováno v: Journal of Experimental Medicine
Journal of Experimental Medicine, Rockefeller University Press, 2018, 215 (9), pp.2289-2310. ⟨10.1084/jem.20170308⟩
Journal of Experimental Medicine, 2018, 215 (9), pp.2289-2310. ⟨10.1084/jem.20170308⟩

International audience; Patients with epidermodysplasia verruciformis (EV) and biallelic null mutations of TMC6 (encoding EVER1) or TMC8 (EVER2) are selectively prone to disseminated skin lesions due to keratinocyte-tropic human β-papillomaviruses (

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8f84fccd94dce744d9f8af1b6a12c3a2
https://doi.org/10.1084/jem.20170308

Homozygous NLRP1 gain-of-function mutation in siblings with a syndromic form of recurrent respiratory papillomatosis

Autor: Sarah Jill De Jong, Sofie De Schepper, Jean-Laurent Casanova, Jutte van der Werff ten Bosch, David Hum, Emmanuelle Jouanguy, Franklin L. Zhong, Vincent R. Bonagura, Scott Drutman, Nicholas Hernandez, David Creytens, Gérard Orth, Katrien Bonte, Andy Wullaert, Franck Rapaport, Filomeen Haerynck, Laurent Abel, Serkan Belkaya, Xavier Bossuyt, Vivien Béziat, Lazaro Lorenzo-Diaz, Bruno Reversade, Simon Tavernier

Publikováno v: Proceedings of the National Academy of Sciences of the United States of America, 116(38), 19055-19063. National Academy of Sciences
Proceedings of the National Academy of Sciences of the United States of America

Juvenile-onset recurrent respiratory papillomatosis (JRRP) is a rare and debilitating childhood disease that presents with recurrent growth of papillomas in the upper airway. Two common human papillomaviruses (HPVs), HPV-6 and -11, are implicated in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7ccc719080bb3cc3a6a38e5db3e92583
https://pure.amc.nl/en/publications/homozygous-nlrp1-gainoffunction-mutation-in-siblings-with-a-syndromic-form-of-recurrent-respiratory-papillomatosis(16988276-9a9b-4487-b87c-baf543687e04).html

The unfolded protein response genes in human osteoarthritic chondrocytes: PERK emerges as a potential therapeutic target

Autor: Jean-Pierre Pelletier, David Hum, Ginette Tardif, Johanne Martel-Pelletier, Mohit Kapoor, Hassan Fahmi, Ying-Hua Li

Publikováno v: Arthritis Research & Therapy

Background The unfolded protein response (UPR) is activated following an endoplasmic reticulum (ER) stress. The aim of this study was to investigate the global expression of UPR genes in human OA chondrocytes in induced (I)-UPR conditions, and to exp

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9f67d7288fa85ee1a069c205fdcffebc
https://doi.org/10.1186/s13075-016-1070-6