Publications related to Alpha-1 antitrypsin deficiency

Alpha1-antitrypsin improves survival in murine abdominal sepsis model by decreasing inflammation and sequestration of free heme

Background Excessive inflammation, hemolysis, and accumulation of labile heme play an essential role in the pathophysiology of multi-organ dysfunction syndrome (MODS) in sepsis. Alpha1-antitrypsin (AAT), an acute phase protein with heme binding capacity, i ...

Frontiers Media Sa2024

Boosted Pro-Inflammatory Activity in Human PBMCs by Lipopolysaccharide and SARS-CoV-2 Spike Protein Is Regulated by alpha-1 Antitrypsin

Florian Maria Wurm, Divor Kiseljak

For the treatment of severe COVID-19, supplementation with human plasma-purified alpha-1 antitrypsin (AAT) to patients is currently considered. AAT inhibits host proteases that facilitate viral entry and possesses broad anti-inflammatory and immunomodulato ...

MDPI2021

The Delivery of alpha 1-Antitrypsin Therapy Through Transepidermal Route: Worthwhile to Explore

Florian Maria Wurm

Human alpha 1-antitrypsin (AAT) is an abundant acute phase glycoprotein expressing anti-protease and immunomodulatory activities, and is used as a biopharmaceutical to treat patients with inherited AAT deficiency. The pleiotropic properties of AAT provide ...

FRONTIERS MEDIA SA2020

Human proteinase 3 resistance to inhibition extends to alpha-2 macroglobulin

Marc Moniatte

Polymorphonuclear neutrophils contain at least four serine endopeptidases, namely neutrophil elastase (NE), proteinase 3 (PR3), cathepsin G (CatG), and NSP4, which contribute to the regulation of infection and of inflammatory processes. In physiological co ...

2020

ER-to-lysosome-associated degradation of proteasome-resistant ATZ polymers occurs via receptor-mediated vesicular transport

Maurizio Molinari, Paolo Paganetti

Maintenance of cellular proteostasis relies on efficient clearance of defective gene products. For misfolded secretory proteins, this involves dislocation from the endoplasmic reticulum (ER) into the cytosol followed by proteasomal degradation. However, po ...

2018

Bilateral cortical representation of tactile roughness

Silvestro Micera, Fiorenzo Artoni

Roughness is the most important feature for texture discrimination. Here we investigate how the bilateral cortical representation of touch is modulated by tactile roughness by analyzing the neural responses elicited by stimuli with various coarseness level ...

2018

Non-invasive diagnosis and metabolic consequences of congenital portosystemic shunts in C57BL/6 J mice

Hongxia Lei, Ana Francisca Leal Silva Soares

This study demonstrates the suitability of magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) for the imaging of congenital portosystemic shunts (PSS) in mice, a vascular abnormality in which mesenteric blood bypasses the liver and i ...

2018

Visualization of hepatic arteries with 3D ultrasound during intra-arterial therapies

Anaïs Laure Marie-Thérèse Badoual, François Michaud, Alexandre Bigot

Liver cancer represents the second most common cause of cancer-related mortality worldwide. The prognosis is poor with an overall mortality of 95%. Moreover, most hepatic tumors are unresectable due to their advanced stage at discovery or poor underlying l ...

Spie-Int Soc Optical Engineering2016

Malectin Participates in a Backup Glycoprotein Quality Control Pathway in the Mammalian ER

Maurizio Molinari, Riccardo Bernasconi

Malectin is a conserved, endoplasmic reticulum (ER)-resident lectin that recognizes high mannose oligosaccharides displaying terminal glucose residues. Here we show that Malectin is an ER stress-induced protein that selectively associates with glycopolypep ...

Public Library of Science2011