A5006558102- RNA regulation and disease
- RNA Research and Splicing
- Skin and Cellular Biology Research
- interferon and immune responses
- Cellular transport and secretion
- Viral Infections and Immunology Research
- Endoplasmic Reticulum Stress and Disease
Leiden University Medical Center
2022-2024
Leiden University
2021
RIG-I-like receptors (RLRs) are cytoplasmic RNA sensors that promote type I and III interferon (IFN) production in response to ligands of viral or endogenous origin. The RLR pathway is tightly regulated by dynamic post-translational modifications, including ubiquitination. Huwe1 a HECT domain-containing giant ubiquitin E3 ligase has not been implicated the IFN pathway. Here, we investigated whether required for induction downstream RLRs. We demonstrate loss severely attenuates expression...
Proteotoxic stress causes profound endoplasmic reticulum (ER) membrane remodeling into a perinuclear quality control compartment (ERQC) for the degradation of misfolded proteins. Subsequent return to homeostasis involves clearance ERQC by endolysosomes. However, factors that ER integrity and dynamics remain unclear. Here, we identify vimentin intermediate filaments as anchors We show engage ER-embedded RING finger protein 26 (RNF26) at C-terminus its domain. This restricts RNF26 subdomains...
Abstract Vault RNAs (vtRNAs) are a family of four small non-coding (ncRNAs) that ubiquitously expressed in many eukaryotes and regulate multiple cellular pathways. Their expression is increased upon infection with various DNA RNA viruses. This suggests they either co-opted by the virus to aid replication or function as an antiviral restriction factor. However, their precise molecular remains unclear. Here, we show picornaviruses, alphaviruses, beta-coronaviruses broadly enhances vtRNA...
Abstract Compartmentalization of organelles in space and time affects their functional state enables higher order regulation essential cellular processes. How organellar residence is maintained a defined area the cell remains poorly understood. In this study, we uncover new role for intermediate filaments maintenance architecture dynamics, which executed through connection between Vimentin ER-embedded ubiquitin ligase ring finger protein 26 (RNF26). While function RNF26 promotes perinuclear...
Abstract RNA editing by the enzyme Adenosine Deaminase Acting on 1 (ADAR1) is an important mechanism which cells avoid innate immune responses to some endogenous RNAs. In ADAR1-deficient cells, unedited self RNAs can form base-paired structures that resemble viral and inadvertently activate antiviral pathways lead induction of type I interferon (IFN). Rare mutations in ADAR1 cause Aicardi-Goutières Syndrome (AGS), a severe childhood autoinflammatory syndrome characterized chronic excessive...