MYELODYSPLASTIC SYNDROME
|
0.400 |
FusionGene
|
group |
ORPHANET |
|
|
|
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
3q26.2/EVI1 rearrangements resulting in EVI1 overexpression play an important role in leukemogenesis and are associated with treatment resistance and a poorer prognosis in patients with acute myeloid leukemia, myelodysplastic syndrome, chronic myeloid leukemia and BCR-ABL negative myeloproliferative neoplasms.
|
29288910 |
2018 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
EVI1 is an oncoprotein inappropriately expressed in acute myeloid leukemia and myelodysplastic syndrome cells.
|
18655152 |
2008 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
EVI1 RNA was expressed in 29% of 34 (95% confidence interval, 20% to 50%) patients with the MDS subtypes refractory anemia (RA), refractory anemia with excess blasts (RAEB), or refractory anemia with excess blasts in transformation (RAEB-T).
|
8049440 |
1994 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
EVI-1 expression was also detected in a subset of acute myeloid leukaemias (AMLs) and myelodysplasia.
|
8932329 |
1996 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
EVI1, located at chromosome band 3q26, encodes a 1051 amino acid zinc finger protein inappropriately expressed in the leukemic cells of 2-5% of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) patients.
|
9067573 |
1997 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
A high MEBE expression score is an unfavorable prognostic marker in MDS and is associated with an increased risk for progression to AML.
|
22488406 |
2012 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Abnormal expression of the Evi-1 gene and overexpression of MDS1-Evi-1 gene may play a role in the pathogenesis or progression of MDS and post-MDS AML.
|
11721451 |
1999 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
Activation of the Evi-1 gene was first described to be associated with the transformation of murine myeloid leukaemias and has previously been detected in cases of human acute myeloid leukaemia (AML) and chronic myeloid leukaemia (CML) in blast crises and in myelodysplastic syndromes.
|
9432037 |
1997 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
Because of the transcriptional activation of the EVI1 family genes in both t(1;3)(p36;q21)-positive MDS/AML and 3q21q26 syndrome, it is suggested that they share a common molecular mechanism for the leukemogenic transformation of the cells.
|
11050005 |
2000 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Both low/high risk MDS may benefit significantly from therapy with ATO/thalidomide, and those with high pre-therapy EVI1 expression may be uniquely sensitive.
|
15203277 |
2004 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Constitutive expression of Evi-1 in hematopoietic cells, which is caused by retroviral insertions or chromosomal translocations and inversions, is closely associated with myelogenous leukemias and myelodysplastic syndromes in mice and humans.
|
10641791 |
1999 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Development and progression of MDS to acute myeloid leukemia is suggested to be a multistep alteration to hematopoietic stem cells consisting of class I and class II alterations: the former targeting genes that are involved in signal transduction (e.g., FLT3, RAS and KIT), whereas the latter affect transcription factors (e.g., RUNX, RARA, EVI1 and WT1).
|
20222800 |
2010 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
Biomarker
|
group |
BEFREE |
Development of a dual-color, double fusion FISH assay to detect RPN1/EVI1 gene fusion associated with inv(3), t(3;3), and ins(3;3) in patients with myelodysplasia and acute myeloid leukemia.
|
20556821 |
2010 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
Ecotropic viral integration site-1 (EVI1) and myelodysplastic syndrome 1 (MDS1) are located at the center of this region, and their DNA copy number increases are associated with at least 5-fold increased RNA transcript levels in 83% and 98% of advanced ovarian cancers, respectively.
|
17409414 |
2007 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
In a mouse bone marrow transplantation model, a RUNX1 mutant, D171N, was shown to collaborate with Evi1 in the development of MDSs; however, this is rare in humans.
|
23471304 |
2013 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
In contrast to previous studies in AML and MDS, the pattern of EVI-1 expression suggests it may facilitate rather than inhibit myeloid differentiation during ATRA treatment.
|
9009083 |
1997 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
In contrast, EVI-1 is barely expressed in normal hematopoietic cells, but it is overexpressed in chronic myelocytic leukemia in blastic crisis and myelodysplastic syndrome-derived leukemia.
|
15156182 |
2004 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
In summary, the results show that the defects in the erythroid development in a subpopulation of patients with myelodysplasia is localized at an early stage of the erythroid differentiation and is associated with the persistent expression of the CD34 antigen and, in some cases, with the expression of Evi-1.
|
8695798 |
1996 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Inducible expression of EVI1 in human myeloid cells causes phenotypes consistent with its role in myelodysplastic syndromes.
|
19605700 |
2009 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
Interestingly, among integration sites identified, Evi1 seemed to collaborate with an AML1 mutant harboring a point mutation in the Runt homology domain (D171N) to induce MDS/AML with an identical phenotype characterized by marked hepatosplenomegaly, myeloid dysplasia, leukocytosis, and biphenotypic surface markers.
|
18192504 |
2008 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
AlteredExpression
|
group |
BEFREE |
Our results suggest that the leukemogenic role of EVI1 expression may differ between post-MDS AML and leukemia, with EVI1 expression associated with a 3q26 abnormality.
|
7780155 |
1995 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
RUNX1-EVI1 is a chimeric gene generated by t(3;21)(q26;q22) observed in patients with aggressive transformation of myelodysplastic syndrome or chronic myelogenous leukemia.
|
19016745 |
2008 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
Biomarker
|
group |
BEFREE |
The EVI1 gene may be expressed through at least two pathways in hematologic malignancies; one is related to chromosomal changes at 3q26, while the other is related to myelodysplasia regardless of chromosomal changes at 3q26 region.
|
9031072 |
1996 |
MYELODYSPLASTIC SYNDROME
|
0.400 |
GeneticVariation
|
group |
BEFREE |
The t(3;21)(q26.2;q22) translocation is a rare chromosomal abnormality exhibited almost exclusively in therapy-related myelodysplastic syndrome/acute myeloid leukemia (t-MDS/AML) or in the blastic crisis phase of chronic myelogenous leukemia, which results in the fusion of the runt related transcription factor 1 (<i>RUNX1</i>, also called <i>AML1</i>) gene at 21q22 to the myelodysplasia syndrome 1 (<i>MDS1</i>)-ecotropic virus integration site 1 (<i>EVI1</i>) complex locus (<i>MECOM</i>) at 3q26.2, generating various fusion transcripts, including <i>AML1/MDS1/EVI1</i> (<i>AME</i>).
|
28693140 |
2017 |