Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms.
NOTCH
cancer-driven genes
germline mutations
malignant castration-resistant prostatic cells
medullary thyroid carcinoma
mutational mechanism
neuroendocrine neoplasms
pancreatic NET
small bowel NET
small cell lung carcinoma
Journal
Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588
Informations de publication
Date de publication:
22 Aug 2019
22 Aug 2019
Historique:
received:
10
07
2019
revised:
13
08
2019
accepted:
20
08
2019
entrez:
25
8
2019
pubmed:
25
8
2019
medline:
25
8
2019
Statut:
epublish
Résumé
Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare malignancies, mainly originating from hormone-secreting cells, which are widespread in human tissues. The identification of mutations in ATRX/DAXX genes in sporadic NENs, as well as the high burden of mutations scattered throughout the multiple endocrine neoplasia type 1 (MEN-1) gene in both sporadic and inherited syndromes, provided new insights into the molecular biology of tumour development. Other molecular mechanisms, such as the NOTCH signalling pathway, have shown to play an important role in the pathogenesis of NENs. NOTCH receptors are expressed on neuroendocrine cells and generally act as tumour suppressor proteins, but in some contexts can function as oncogenes. The biological heterogeneity of NENs suggests that to fully understand the role and the potential therapeutic implications of gene mutations and NOTCH signalling in NENs, a comprehensive analysis of genetic alterations, NOTCH expression patterns and their potential role across all NEN subtypes is required.
Identifiants
pubmed: 31443481
pii: jcm8091277
doi: 10.3390/jcm8091277
pmc: PMC6780206
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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