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Università della Svizzera italiana

Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice

Volpi, Vera G. ; Ferri, Cinzia ; Fregno, Ilaria ; Del Carro, Ubaldo ; Bianchi, Francesca ; Scapin, Cristina ; Pettinato, Emanuela ; Solda, Tatiana ; Feltri, M. Laura ; Molinari, Maurizio ; Wrabetz, Lawrence ; D’Antonio, Maurizio

In: PLOS genetics, 2019, vol. 15, no. 4, p. e1008069

In the peripheral nervous system (PNS) myelinating Schwann cells synthesize large amounts of myelin protein zero (P0) glycoprotein, an abundant component of peripheral nerve myelin. In humans, mutations in P0 cause the demyelinating Charcot-Marie-Tooth 1B (CMT1B) neuropathy, one of the most diffused genetic disorders of the PNS. We previously showed that several mutations, such as the...

Università della Svizzera italiana

Quality control mechanisms of protein biogenesis : proteostasis dies hard

Bergmann, Timothy Jan ; Brambilla Pisoni, Giorgia ; Molinari, Maurizio

In: AIMS biophysics, 2016, vol. 3, no. 4, p. 456-478

The biosynthesis of proteins entails a complex series of chemical reactions that transform the information stored in the nucleic acid sequence into a polypeptide chain that needs to properly fold and reach its functional location in or outside the cell. It is of no surprise that errors might occur that alter the polypeptide sequence leading to a non-functional proteins or that impede delivery...

Università della Svizzera italiana

Post ER quality control : a role for UDP-glucose : glycoprotein glucosyl transferase and p97

Fregno, Ilaria ; Molinari, Maurizio

In: Journal of rare diseases research & treatment, 2016, vol. 1, no. 1, p. 40-42

Human proteinopathies are diseases caused by the expression of defective gene products. In some cases, these diseases involve the degradation of mutant but otherwise functional proteins by the quality control system of the secretory pathway. Our recent study identified two proteins that play a role in post-endoplasmic reticulum (ER) quality control and are potential targets for therapeutic ...

Università della Svizzera italiana

Proteostasis : bad news and good news from the endoplasmic reticulum

Noack, Julia ; Brambilla Pisoni, Giorgia ; Molinari, Maurizio

In: Swiss medical weekly, 2014, vol. 144, p. w14001

The endoplasmic reticulum (ER) is an intracellular compartment dedicated to the synthesis and maturation of secretory and membrane proteins, totalling about 30% of the total eukaryotic cells proteome. The capacity to produce correctly folded polypeptides and to transport them to their correct intra- or extracellular destinations relies on proteostasis networks that regulate and balance the...

Università della Svizzera italiana

Endoplasmic reticulum turnover : ER-phagy and other flavors in selective and non-selective ER clearance

Fregno, Ilaria ; Molinari, Maurizio

In: F1000Research, 2018, vol. 7, p. 454

The endoplasmic reticulum (ER) is a highly dynamic organelle in eukaryotic cells. It is deputed to lipid and protein biosynthesis, calcium storage, and the detoxification of various exogenous and endogenous harmful compounds. ER activity and size must be adapted rapidly to environmental and developmental conditions or biosynthetic demand. This is achieved on induction of thoroughly studied...

Università della Svizzera italiana

Unconventional use of LC3 by coronaviruses through the alleged subversion of the ERAD tuning pathway

Reggiori, Fulvio ; De Haan, Cornelis A.M. ; Molinari, Maurizio

In: Viruses, 2011, vol. 3, no. 9, p. 1610-1623

Pathogens of bacterial and viral origin hijack pathways operating in eukaryotic cells in many ways in order to gain access into the host, to establish themselves and to eventually produce their progeny. The detailed molecular characterization of the subversion mechanisms devised by pathogens to infect host cells is crucial to generate targets for therapeutic intervention. Here we review recent...

Università della Svizzera italiana

Chronic delivery of antibody fragments using immunoisolated cell implants as a passive vaccination tool

Belaunzaran, Osiris Marroquin ; Cordero, Maria Isabel ; Setola, Veronica ; Bianchi, Siro ; Galli, Carmela ; Bouche, Nicolas ; Mlynarik, Vladimir ; Gruetter, Rolf ; Sandi, Carmen ; Bensadoun, Jean-Charles ; Molinari, Maurizio ; Aebischer, Patrick

In: Plos one, 2011, vol. 6, no. 4, p. e18268

Background: Monoclonal antibodies and antibody fragments are powerful biotherapeutics for various debilitating diseases. However, high production costs, functional limitations such as inadequate pharmacokinetics and tissue accessibility are the current principal disadvantages for broadening their use in clinic. Methodology and Principal Findings: We report a novel method for the long-term ...

Università della Svizzera italiana

Malectin participates in a backup glycoprotein quality control pathway in the mammalian ER

Galli, Carmela ; Bernasconi, Riccardo ; Soldà, Tatiana ; Calanca, Verena ; Molinari, Maurizio

In: Plos one, 2011, vol. 6, no. 1, p. e16304

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 glycopolypeptides without affecting their entry and their retention in the Calnexin chaperone system. Analysis of the obligate Calnexin client...

Università della Svizzera italiana

Cyclosporine A-sensitive, cyclophilin B-dependent endoplasmic reticulum-associated degradation

Bernasconi, Riccardo ; Soldà, Tatiana ; Galli, Carmela ; Pertel, Thomas ; Luban, Jeremy ; Molinari, Maurizio

In: Plos one, 2010, vol. 5, no. 9, p. e13008

Peptidyl-prolyl cis/trans isomerases (PPIs) catalyze cis/trans isomerization of peptide bonds preceding proline residues. The involvement of PPI family members in protein refolding has been established in test tube experiments. Surprisingly, however, no data is available on the involvement of endoplasmic reticulum (ER)-resident members of the PPI family in protein folding, quality control or...

Università della Svizzera italiana

The protein disulfide isomerase ERp57 regulates the steady-state levels of the prion protein

Torres, Mauricio ; Medinas,Danilo B. ; Matamala, José Manuel ; Woehlbier, Ute ; Cornejo, Víctor Hugo ; Solda, Tatiana ; Andreu, Catherine ; Rozas, Pablo ; Matus, Soledad ; Muñoz, Natalia ; Vergara, Carmen ; Cartier, Luis ; Soto, Claudio ; Molinari, Maurizio ; Hetz, Claudio

In: The journal of biological chemistry, 2015, vol. 290, no. 39, p. 23631-23645

Although the accumulation of a misfolded and protease-resistant form of the prion protein (PrP) is a key event in Prion pathogenesis, the cellular factors involved in its folding and quality control are poorly understood. PrP is a glycosylated and disulfide-bonded protein synthesized at the endoplasmic reticulum (ER). The ER foldase ERp57 (also known as Grp58) is highly expressed in the brain...