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ProteomicsDysregulation of protein homeostasis by mutant UBA1 in VEXAS syndrome
VEXAS is a hematopoietic disorder characterized by hyperinflammation,high mortality, and mutations at methionine 41 (M41) in the E1 ubiquitin enzyme, UBA1. Here, we developed ahuman model of VEXAS by engineering the male THP1 cell line to express the UBA1-M41V mutation. We found that UBA1-M41V cells...
ORGANISM(S): Homo sapiens (Human) 
2025-08-04 | PXD063067 | Pride
Human Vexas 
ProteomicsDysregulation of protein homeostasis by mutant UBA1 in VEXAS syndrome
VEXAS is a hematopoietic disorder characterized by hyperinflammation,high mortality, and mutations at methionine 41 (M41) in the E1 ubiquitin enzyme, UBA1. Here, we developed ahuman model of VEXAS by engineering the male THP1 cell line to express the UBA1-M41V mutation. We found that UBA1-M41V cells...
ORGANISM(S): Homo sapiens (Human) 
2025-08-04 | PXD063068 | Pride
Vexas Uba1 
TranscriptomicsCytotoxic effects of Bortezomib in Myelodysplastic syndrome/Acute Myeloid Leukemia depend on autophagy-mediated lysosomal degradation of TRAF6 and repression of PSMA1
Bortezomib (VelcadeM-BM-)) is widely used for the treatment of various human cancers, however, its mechanisms of action are not fully understood, particularly in myeloid malignancies. Bortezomib is a selective and reversible inhibitor of the proteasome. Paradoxically, we find that Bortezomib induces...
ORGANISM(S): Homo sapiens 
2012-06-14 | E-GEOD-38519 | biostudies-arrayexpress
TranscriptomicsMyeloid malignancies with chromosome 5q deletions acquire a dependency on an intrachromosomal NF-M-NM-:B gene network
Chromosome 5q deletions (del(5q)) are common in high-risk (HR) Myelodysplastic Syndrome (MDS) and Acute Myeloid Leukemia (AML); however, the gene regulatory networks that sustain these aggressive diseases are unknown. Reduced miR-146a expression in del(5q) HR-MDS/AML and miR-146a-/- hematopoietic st...
ORGANISM(S): Homo sapiens 
2014-08-23 | E-GEOD-60649 | biostudies-arrayexpress
TranscriptomicsLoss of Tifab, a del(5q) MDS gene, alters hematopoiesis through derepression of Toll-like receptor/TRAF6 signaling
TRAF-interacting protein with forkhead-associated domain B (TIFAB) is a haploinsufficient gene in del(5q) Myelodysplastic syndrome (MDS). Hematopoietic-specific deletion of Tifab results in progressive bone marrow (BM) and blood defects, including skewed hematopoietic stem/progenitor cells (HSPC) pr...
ORGANISM(S): Mus musculus 
2015-09-12 | E-GEOD-72936 | biostudies-arrayexpress
TranscriptomicsTargeting IRAK1 as a therapeutic approach for Myelodysplastic Syndrome
Myelodysplastic Syndromes (MDS) result from expansion of defective hematopoietic stem/progenitor clones. There is an urgent need to develop targeted therapies capable of eliminating the defective MDS clones. We identified that IRAK1, an immune modulating kinase, is overexpressed and hyperactivated i...
ORGANISM(S): Homo sapiens 
2013-04-25 | E-GEOD-46346 | biostudies-arrayexpress
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