|
T-Cell Prolymphocytic Leukemia
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
We formulate a multi-dimensional model of T-PLL pathogenesis centered around a unique combination of TCL1 overexpression with damaging ATM aberrations as initiating core lesions.
|
29449575 |
2018 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
The frequent MYC, ATM, and TCL1A alterations identified support that these genes are integral to the pathogenesis of T-PLL.
|
25310835 |
2014 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
The Ataxia Telangiectasia Mutated (ATM) gene is frequently inactivated in lymphoid malignancies such as chronic lymphocytic leukemia (CLL), T-prolymphocytic leukemia (T-PLL), and mantle cell lymphoma (MCL) and is associated with defective apoptosis in response to alkylating agents and purine analogues.
|
20739657 |
2010 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
T-cell prolymphocytic leukemia is a rare aggressive lymphoproliferative disease with a mature T-cell phenotype and characteristic genomic lesions such as inv(14)(q11q34), t(14;14)(q11;q32) or t(X;14)(q28;q11), mutation of the ATM gene on chromosome 11 and secondary alterations such as deletions of chromosome 8p and duplications of 8q.
|
19278963 |
2009 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
T-cell prolymphocytic leukemia (T-PLL) is consistently associated with inactivation of the ATM gene and chromosomal re-arrangements leading to an overexpression of MTCP1/TCL1 oncoproteins.
|
18073348 |
2008 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
Intriguingly, recent studies have strongly implicated ATM in the pathogenesis of T-PLL as a tumor suppressor gene, because biallelic inactivation of ATM is frequently observed in this disease; however, translocations involving 11q23 have rarely been reported in T-PLL.
|
14499692 |
2003 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Moreover, somatic ATM mutations have been identified in T-cell prolymphocytic leukemia, mantle cell lymphoma, and B-cell chronic lymphocytic leukemia.
|
12149228 |
2002 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
Because some data suggest ATM is frequently rearranged in T-PLL, it was decided to investigate such rearrangements in detail by cloning breakpoints.
|
11821961 |
2002 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
Frequent ATM inactivation was confirmed in three sporadic lymphoid tumours of mature phenotype: T cell prolymphocytic leukaemia (T-PLL), B-cell chronic lymphocytic leukaemia (B-CLL) and mantle cell lymphoma (MCL).
|
12400598 |
2002 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
The presence of inactivating mutations, together with the deletion of the normal copy of the ATM gene in some patients with T-PLL, B-CLL, and MCL, establishes somatic inactivation of the ATM gene in the pathogenesis of lymphoid malignancies, and strongly suggests that ATM functions as a tumour suppressor.
|
11429421 |
2001 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
The putative tumor suppressor genes have remained unrevealed until recently, when the ATM gene was found to carry mutations in cases with deletion in B-CLL, MCL and T-PLL.
|
11426547 |
2001 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
ATM mutations are also involved in the development of sporadic human cancers such as T-cell prolymphocytic leukemia and B-cell chronic lymphocytic leukemia.
|
10738255 |
2000 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
As mutations of the Ataxia Telangiectasia (A-T) gene, ATM, are frequent in T-PLL and as ATM seems to act as a tumour suppressor through a mechanism involving V(D)J recombination, we examined V(D)J recombination in T-PLL.
|
11054065 |
2000 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
We report a case of T-PLL with t(6;11)(q21;q23) as the sole chromosomal abnormality and suggest that the cytogenetically identified translocation also implicates the ATM gene.
|
10347553 |
1999 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
For example, the gene ataxia telangiectasia mutated (ATM) has recently been shown to be a tumour suppressor gene in T-cell prolymphocytic leukaemia, and there is increasing evidence that individuals with one mutated ATM or Nijmegen breakage syndrome (NBS1) allele have an increased predisposition to cancer.
|
10203748 |
1999 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
ATM does seem to act as a classic tumor suppressor gene in T-prolymphocytic leukemia, and LOH at the ATM locus is a common event in some tumor types, suggesting a general role for ATM in cancer.
|
9699683 |
1998 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
ATM is usually rearranged in T-cell prolymphocytic leukaemia.
|
9488043 |
1998 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
Biomarker
|
disease |
BEFREE |
The absence of somatic nucleotide changes in ATM in T-ALL as compared with T-PLL suggests a distinct pattern of genetic events in the development of the two leukemias.
|
9622061 |
1998 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
AlteredExpression
|
disease |
BEFREE |
However, in five T-PLLs with LOH of the 11q22-23 region, Western blot analysis showed either undetectable (3 cases) or decreased levels (1 case) of ATM protein, whereas ATM was present at high levels in cases without LOH.
|
9573030 |
1998 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
By analysing tumour DNA from patients with sporadic T-cell prolymphocytic leukaemia (T-PLL), a rare clonal malignancy with similarities to a mature T-cell leukaemia seen in A-T, we demonstrate a high frequency of ATM mutations in T-PLL.
|
9288106 |
1997 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GeneticVariation
|
disease |
BEFREE |
Biallelic mutations in the ATM gene in T-prolymphocytic leukemia.
|
9334731 |
1997 |
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
CausalMutation
|
disease |
CLINVAR |
|
|
|
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
CausalMutation
|
disease |
CGI |
|
|
|
|
T-Cell Prolymphocytic Leukemia
|
0.500 |
GenomicAlterations
|
disease |
CGI |
|
|
|