Publications de Béatrice Kunz

Injury-induced intestinal stem cell renewal requires capillary morphogenesis gene 2

Béatrice Kunz, Olivier Burri, Françoise Gisou van der Goot Grunberg, Nathalie Brandenberg, Julien Léonard Duc, Romain Guiet, Audrey Geneviève Chuat, Lucie Josepha Marie Bracq

Patients with the rare genetic disorder Hyaline Fibromatosis Syndrome (HFS) often succumb before 18 months of age due to severe diarrhea. As HFS is caused by loss-of-function mutations in the gene encoding capillary morphogenesis gene 2 (CMG2), these sympt ...

Springer Science and Business Media LLC2025

Dynamics of CLIMP-63 S-acylation control ER morphology

Kathryn Hess Bellwald, Béatrice Kunz, Graham Knott, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Vassily Hatzimanikatis, Patrick Alain Sandoz, Robin Alexander Denhardt-Eriksson, Sylvia Ho, Luciano Andres Abriata, Catherine Maclachlan, Gard Spreemann

A key player in the formation of endoplasmic reticulum sheets is CLIMP-63, but mechanistic details remained elusive. Here authors combined cellular experiments and mathematical modelling to show that S-acylation of CLIMP-63 regulates its function by mediat ...

Nature Portfolio2023

SARS-CoV-2 hijacks a cell damage response, which induces transcription of a more efficient Spike S-acyltransferase

Béatrice Kunz, Didier Trono, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Audrey Geneviève Chuat, Francisco De Magalhães Sarmento R De Mesquita, Lucie Josepha Marie Bracq, Joana Carlevaro Fita, Nattawadee Panyain

SARS-CoV-2 infection requires Spike protein-mediated fusion between the viral and cellular membranes. The fusogenic activity of Spike depends on its post-translational lipid modification by host S-acyltransferases, predominantly ZDHHC20. Previous observati ...

Nature Research2023

S-acylation controls SARS-CoV-2 membrane lipid organization and enhances infectivity

Béatrice Kunz, Didier Trono, Priscilla Turelli, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Matteo Dal Peraro, Charlène Mireille Raymonde Raclot, Luciano Andres Abriata, Oksana Andrei Sergeeva, Giovanni D'Angelo, Francisco De Magalhães Sarmento R De Mesquita

SARS-CoV-2 virions are surrounded by a lipid bilayer that contains membrane proteins such as spike, responsible for target-cell binding and virus fusion. We found that during SARS-CoV-2 infection, spike becomes lipid modified, through the sequential action ...

Cell Press - Elsevier2021

Ligand Binding to the Collagen VI Receptor Triggers a Talin-to-RhoA Switch that Regulates Receptor Endocytosis

Béatrice Kunz, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Matteo Dal Peraro, Shixu Yan, Luciano Andres Abriata

Capillary morphogenesis gene 2 (CMG2/ANTXR2) is a cell surface receptor for both collagen VI and anthrax toxin. Biallelic loss-of-function mutations in CMG2 lead to a severe condition, hyaline fibromatosis syndrome (HFS). We have here dissected a network o ...

Cell Press - Elsevier2020

CMG2/ANTXR2 regulates extracellular collagen VI which accumulates in hyaline fibromatosis syndrome

Béatrice Kunz, Jérôme Bürgi, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Julie Deuquet Ariosa, Alessandra Piersigilli

Loss-of-function mutations in capillary morphogenesis gene 2 (CMG2/ANTXR2), a transmembrane surface protein, cause hyaline fibromatosis syndrome (HFS), a severe genetic disorder that is characterized by large subcutaneous nodules, gingival hypertrophy and ...

Springer Nature2017

Ubiquitin-dependent folding of the Wnt signaling coreceptor LRP6

Béatrice Kunz, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Michal Feldman Elmalam, Elsa Anne Perrody

Many membrane proteins fold inefficiently and require the help of enzymes and chaperones. Here we reveal a novel folding assistance system that operates on membrane proteins from the cytosolic side of the endoplasmic reticulum (ER). We show that folding of ...

Elife Sciences Publications Ltd2016

Palmitoylated calnexin is a key component of the ribosome-translocon complex

Thomas Lemmin, Béatrice Kunz, Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami, Matteo Dal Peraro, Sanja Blaskovic

A third of the human genome encodes N-glycosylated proteins. These are co-translationally translocated into the lumen/membrane of the endoplasmic reticulum (ER) where they fold and assemble before they are transported to their final destination. Here, we s ...

2012

Engineering substrate specificity of O6-alkylguanine-DNA alkyltransferase for specific protein labeling in living cells

Hugues Jaccard, Kai Johnsson, Alexandre Juillerat, India Christina Sielaff, Béatrice Kunz, Christian Heinis

Fusion proteins of human O6-alkylguanine-DNA alkyltransferase (AGT) can be specifically labeled with a wide variety of synthetic probes in mammalian cells; this makes them an attractive tool for studying protein function. However, to avoid undesired labeli ...

2005