2
Mechanism
VEXAS Syndrome
Preface
1
Introduction
2
Mechanism
3
Independent Mechanisms of Inflammation and Myeloid Bias in VEXAS Syndrome
4
Variants of UBA1
5
Treatments of VEXAS
6
SIR9900 in VEXAS Syndrome
7
Summary
References
Table of contents
2.1
Activation of the unfolded protein response in mutant HSCs
2.2
Mutant cells are highly inflamed in VEXAS compared to wild-type cells
2.3
Mechanisms of hematopoietic clonal dominance in VEXAS syndrome
2
Mechanism
UBA1 Mutations Result in
Loss of UBA1b
and
Gain of a New Cytoplasmic Isoform, UBA1c
2.1
Activation of the unfolded protein response in mutant HSCs
2.2
Mutant cells are highly inflamed in VEXAS compared to wild-type cells
2.3
Mechanisms of hematopoietic clonal dominance in VEXAS syndrome
1
Introduction
3
Independent Mechanisms of Inflammation and Myeloid Bias in VEXAS Syndrome