Proteomics

Dataset Information

0

RIGI autoimmune variants conformational changes


ABSTRACT: HDX-MS investigation on RIG-I variants and their conformational change upon recognition of self and nonself RNA recognition

INSTRUMENT(S): Orbitrap Fusion

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Fibroblast

DISEASE(S): Syndrome

SUBMITTER: Song Bin  

LAB HEAD: Zheng Jie

PROVIDER: PXD031831 | Pride | 2022-08-12

REPOSITORIES: Pride

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Publications

A loosened gating mechanism of RIG-I leads to autoimmune disorders.

Lei Yixuan Y   Fei Panyu P   Song Bin B   Shi Wenjia W   Luo Cheng C   Luo Dahai D   Li Dan D   Chen Wei W   Zheng Jie J  

Nucleic acids research 20220601 10


DDX58 encodes RIG-I, a cytosolic RNA sensor that ensures immune surveillance of nonself RNAs. Individuals with RIG-IE510V and RIG-IQ517H mutations have increased susceptibility to Singleton-Merten syndrome (SMS) defects, resulting in tissue-specific (mild) and classic (severe) phenotypes. The coupling between RNA recognition and conformational changes is central to RIG-I RNA proofreading, but the molecular determinants leading to dissociated disease phenotypes remain unknown. Herein, we employed  ...[more]

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