Results 61 to 70 of about 98,834 (305)

Angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathies of undetermined significance.

Journal of Allergy and Clinical Immunology: In Practice
BACKGROUND No specific description of monoclonal gammopathies of undetermined significance (MGUS)-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) has been reported yet.
Constance Lahuna, F. Defendi, L. Bouillet, I. Boccon-Gibod, Arsène Mekinian, P. Coppo, H. Adamski, Stephanie Amarger, Guillaume Armengol, M. Aubineau, Beatrice Bibes, C. Blanchard-Delaunay, Gilles Blaison, B. Brihaye, P. Cathébras, Olivier Caubet, Claire Demoreuil, Julien Desblache, François Durupt, Stéphane Gayet, G. Gondran, J. Hadjadj, Galith Kalmi, Gisèle Kanny, M. Lacoste, David Launay, K. Ly, C. Mcavoy, Ludovic Martin, Y. Ollivier, Fabien Pelletier, Aylsa Robbins, Damien Roos-Weil, O. Fain, D. Gobert   +34 more
semanticscholar   +1 more source

Practical Management of C1 Inhibitor Deficiency

Actas Dermo-Sifiliográficas (English Edition), 2007
C1 inhibitor deficiency is a rare syndrome clinically characterized by recurrent episodes of swelling of subcutaneous tissue or angioedema. It can involve the skin, upper respiratory airways and abdomen. There are two main types: hereditary and acquired. Angioedema can involve practically any part of the cutaneous surface, it can cause lethal laryngeal
J, Pedraz, E, Daudén, A, García-Diez
openaire   +2 more sources

Transferrin receptor 1‐mediated iron uptake supports thermogenic activation in human cervical‐derived adipocytes

FEBS Letters, EarlyView.
In this study, we found that human cervical‐derived adipocytes maintain intracellular iron level by regulating the expression of iron transport‐related proteins during adrenergic stimulation. Melanotransferrin is predicted to interact with transferrin receptor 1 based on in silico analysis.
Rahaf Alrifai, Mizuki Seo, Gyath Karadsheh, Fachrur Rizal Mahendra, Máté Á Demény, Ferenc Győry, János András Mótyán, László Fésüs, Endre Kristóf, Rini Arianti   +9 more
wiley   +1 more source

Risk of angioedema following invasive or surgical procedures in HAE type I and II : the natural history

, 2013
Background: Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), leads to unpredictable edema of subcutaneous tissues with potentially fatal complications.
Aygören-Pürsün, Emel, Kreuz, Wolfhart, Martinez Saguer, Inmaculada, Klingebiel, Thomas, Schwabe, Dirk   +4 more
core   +1 more source

Structural insights into an engineered feruloyl esterase with improved MHET degrading properties

FEBS Letters, EarlyView.
A feruloyl esterase was engineered to mimic key features of MHETase, enhancing the degradation of PET oligomers. Structural and computational analysis reveal how a point mutation stabilizes the active site and reshapes the binding cleft, expading substrate scope.
Panagiota Karampa, Konstantinos Makryniotis, Theofani‐Iosifina Sousani, Evangelos Topakas, Vangelis Daskalakis, Maria Dimarogona   +5 more
wiley   +1 more source

Diversity and complexity in neural organoids

FEBS Letters, EarlyView.
Neural organoid research aims to expand genetic diversity on one side and increase tissue complexity on the other. Chimeroids integrate multiple donor genomes within single organoids. Self‐organising multi‐identity organoids, exogenous cell seeding, or enforced assembly of region‐specific organoids contribute to tissue complexity.
Ilaria Chiaradia, Madeline A. Lancaster
wiley   +1 more source

Angioedema

Revista Alergia México, 2016
Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours.
Luisa María Holguín-Gómez, Luz Adriana Vásquez-Ochoa, Ricardo Cardona   +2 more
doaj   +1 more source

Three new Alpha1-Antitrypsin deficiency variants help to define a C-Terminal region regulating conformational change and polymerization

, 2012
Alpha1-antitrypsin (AAT) deficiency is a hereditary disorder associated with reduced AAT plasma levels, predisposing adults to pulmonary emphysema.
Fra Anna M., Fra, Anna M, Anna M. Fra (157302), Scabini, Roberta, Federica Benini, Ferrarotti, Ilaria, L. Corda, Corda, Luciano, Scabini R, E. Miranda, LUISETTI, MAURIZIO, Miranda Elena, Benini, Federica, Corda, L., Benini, F., Medicina D, Luciano Corda, Maurizio Luisetti (142908), Luisa Schiaffonati, I. Ferrarotti, Anna M. Fra, Ilaria Ferrarotti (142893), Luisetti M, Gooptu Bibek, Miranda, E., SCHIAFFONATI, Luisa, Gooptu, Bibekbrata, Anna M Fra, Medicina, Daniela, Elena Miranda, Schiaffonati Luisa, Ferrarotti, I., Medicina, D., R. Ronzoni, Luisetti Maurizio, Daniela Medicina, R. Scabini, L. Schiaffonati, Roberta Scabini (157306), Ferrarotti Ilaria, Ronzoni, R., B. Gooptu, Ferrarotti I, Bibek Gooptu (157303), Luciano Corda (157317), F. Benini, Scabini Roberta, Gasset, M., A. M. Fra, MIRANDA BANOS, MARIA ELENA, Fra, A.M., Ilaria Ferrarotti, Medicina Daniela, FRA, Annamaria, RONZONI, Riccardo, Gooptu B, Daniela Medicina (157321), Ronzoni Riccardo, Benini Federica, Miranda, Elena, D. Medicina, Corda Luciano, Maurizio Luisetti, Benini F, Scabini, R., Corda L, Roberta Scabini, Riccardo Ronzoni (157309), Luisa Schiaffonati (157328), Federica Benini (157312), Riccardo Ronzoni, Gooptu, Bibek, Luisetti, M., Schiaffonati, L., Elena Miranda (21508), Bibek Gooptu, Miranda E   +76 more
core   +1 more source

Hyperosmotic stress induces PARP1‐mediated HPF1‐dependent mono(ADP‐ribosyl)ation

FEBS Letters, EarlyView.
Sorbitol‐induced hyperosmotic stress rapidly induces reversible mono(ADP‐ribosyl)ation (MARylation) on PARP1 without the signs of genotoxic signaling. We show that PARP1 autoMARylation is HPF1 dependent and forms hydroxylamine‐resistant O‐glycosidic linkages.
Anna Georgina Kopasz, Mihály Mérey, Rebeka Vásárhelyi, Ramóna Pék, Victor Imburchia, László Henn, Adrián Kószó, Nicholas D. Lakin, Ivan Ahel, Sébastien Huet, Ágnes Czibula, Gyula Timinszky   +11 more
wiley   +1 more source

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Allergy. European Journal of Allergy and Clinical Immunology, 2016
The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients.
H. Farkas, I. Martínez-Saguer, K. Bork, T. Bowen, T. Craig, M. Frank, A. Germenis, A. Grumach, A. Luczay, L. Varga, A. Zanichelli, W. Aberer, S. Andrejević, Emel Aygoeren‐Pürsün, A. Banerji, N. Bara, M. Baş, J. Bernstein, S. Betschel, J. Björkander, I. Boccon-Gibod, L. Bouillet, M. Bova, H. Boysen, M. Branco-Ferreira, A. Bygum, T. Caballero, M. Cancian, A. Castaldo, S. Christiansen, M. Cicardi, C. Drouet, J. Fabiani, M. Gompels, M. Gonzalez-Quevedo, J. Gooi, R. Gower, Nihal Mete Gökmen, V. Grivcheva-Panovska, M. Guilarte, O. Gülbahar, E. Hack, R. Hakl, G. Harmat, Miloš Jeseňák, S. Jolles, A. Kaplan, C. Katelaris, M. Košnik, K. V. Kőhalmi, I. Leibovich, M. Levi, Henry H. Li, H. Longhurst, W. Lumry, M. Magerl, A. Malbrán, Ludovic Martin, M. Maurer, E. Mihály, D. Moldovan, M. Murdjeva, Imola Nagy, E. W. Nielsen, Sandra Nieto, P. Nordenfelt, Kristine Obtulowitzc, M. Pedrosa, G. Porębski, N. Prior, A. Reshef, M. Riedl, B. Rosenkranz, P. Schmid‐Grendelmeier, Spath Péter, M. Speletas, M. Staevska, M. Stobiecki, M. Triggiani, N. Veszeli, W. Wuillemin, Zhi Xiang, B. Yamamoto, B. Zuraw   +83 more
semanticscholar   +1 more source