PMID- 33066578
OWN - NLM
STAT- MEDLINE
DCOM- 20210225
LR  - 20210225
IS  - 1422-0067 (Electronic)
IS  - 1422-0067 (Linking)
VI  - 21
IP  - 20
DP  - 2020 Oct 14
TI  - Multiple Endocrine Neoplasia Type 1: The Potential Role of microRNAs in the 
      Management of the Syndrome.
LID - 10.3390/ijms21207592 [doi]
LID - 7592
AB  - Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited tumor syndrome, 
      characterized by the development of multiple neuroendocrine tumors (NETs) in a 
      single patient. Major manifestations include primary hyperparathyroidism, 
      gastro-entero-pancreatic neuroendocrine tumors, and pituitary adenomas. In 
      addition to these main NETs, various combinations of more than 20 endocrine and 
      non-endocrine tumors have been described in MEN1 patients. Despite advances in 
      diagnostic techniques and treatment options, which are generally similar to those 
      of sporadic tumors, patients with MEN1 have a poor life expectancy, and the need 
      for targeted therapies is strongly felt. MEN1 is caused by germline heterozygous 
      inactivating mutations of the MEN1 gene, which encodes menin, a tumor suppressor 
      protein. The lack of a direct genotype-phenotype correlation does not permit the 
      determination of the exact clinical course of the syndrome. One of the possible 
      causes of this lack of association could be ascribed to epigenetic factors, 
      including microRNAs (miRNAs), single-stranded non-coding small RNAs that 
      negatively regulate post-transcriptional gene expression. Some miRNAs, and their 
      deregulation, have been associated with MEN1 tumorigenesis. Recently, an 
      extracellular class of miRNAs has also been identified (c-miRNAs); variations in 
      their levels showed association with various human diseases, including tumors. 
      The aim of this review is to provide a general overview on the involvement of 
      miRNAs in MEN1 tumor development, to be used as possible targets for novel 
      molecular therapies. The potential role of c-miRNAs as future non-invasive 
      diagnostic and prognostic biomarkers of MEN1 will be discussed as well.
FAU - Donati, Simone
AU  - Donati S
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Ciuffi, Simone
AU  - Ciuffi S
AUID- ORCID: 0000-0002-1788-4426
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Marini, Francesca
AU  - Marini F
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Palmini, Gaia
AU  - Palmini G
AUID- ORCID: 0000-0002-8376-7404
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Miglietta, Francesca
AU  - Miglietta F
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Aurilia, Cinzia
AU  - Aurilia C
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
FAU - Brandi, Maria Luisa
AU  - Brandi ML
AD  - Department of Experimental and Clinical Biomedical Sciences "Mario Serio", 
      University of Study of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
AD  - Unit of Bone and Mineral Diseases, University Hospital of Florence, Largo Palagi 
      1, 50139 Florence, Italy.
AD  - Fondazione Italiana Ricerca sulle Malattie dell'Osso (FIRMO Onlus), 50141 
      Florence, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20201014
PL  - Switzerland
TA  - Int J Mol Sci
JT  - International journal of molecular sciences
JID - 101092791
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Circulating MicroRNA)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Animals
MH  - Biomarkers, Tumor/blood/*genetics/metabolism
MH  - Circulating MicroRNA/blood/*genetics/metabolism
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*genetics/metabolism/therapy
MH  - Proto-Oncogene Proteins/genetics/metabolism
PMC - PMC7589704
OTO - NOTNLM
OT  - GEP-NETs
OT  - MEN1
OT  - circulating miRNAs
OT  - miRNAs
OT  - non-invasive biomarkers
OT  - parathyroid glands
OT  - personalized medicine
OT  - pituitary gland
COIS- All the authors declare no conflict of interest.
EDAT- 2020/10/18 06:00
MHDA- 2021/02/26 06:00
PMCR- 2020/10/01
CRDT- 2020/10/17 01:04
PHST- 2020/09/24 00:00 [received]
PHST- 2020/10/12 00:00 [revised]
PHST- 2020/10/12 00:00 [accepted]
PHST- 2020/10/17 01:04 [entrez]
PHST- 2020/10/18 06:00 [pubmed]
PHST- 2021/02/26 06:00 [medline]
PHST- 2020/10/01 00:00 [pmc-release]
AID - ijms21207592 [pii]
AID - ijms-21-07592 [pii]
AID - 10.3390/ijms21207592 [doi]
PST - epublish
SO  - Int J Mol Sci. 2020 Oct 14;21(20):7592. doi: 10.3390/ijms21207592.