TP53 in Myelodysplastic Syndromes: Recent Biological and Clinical Findings.
Int J Mol Sci
; 21(10)2020 May 13.
Article
em En
| MEDLINE
| ID: mdl-32414002
ABSTRACT
TP53 dysregulation plays a pivotal role in the molecular pathogenesis of myelodysplastic syndromes (MDS), identifying a subgroup of patients with peculiar features. In this review we report the recent biological and clinical findings of TP53-mutated MDS, focusing on the molecular pathways activation and on its impact on the cellular physiology. In MDS, TP53 mutational status is deeply associated with del(5q) syndrome and its dysregulation impacts on cell cycle, DNA repair and apoptosis inducing chromosomal instability and the clonal evolution of disease. TP53 defects influence adversely the MDS clinical outcome and the treatment response rate, thus new therapeutic approaches are being developed for these patients. TP53 allelic state characterization and the mutational burden evaluation can therefore predict prognosis and identify the subgroup of patients eligible for targeted therapy. For these reasons, in the era of precision medicine, the MDS diagnostic workup cannot do without the complete assessment of TP53 mutational profile.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndromes Mielodisplásicas
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Proteína Supressora de Tumor p53
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Instabilidade Cromossômica
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Terapia de Alvo Molecular
Limite:
Humans
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article