A Comprehensive Structure-Function Study of Neurogenin3 Disease-Causing Alleles during Human Pancreas and Intestinal Organoid Development.
Animals
Basic Helix-Loop-Helix Transcription Factors
/ chemistry
Cell Line
Diarrhea
/ congenital
Human Embryonic Stem Cells
/ metabolism
Humans
Intestinal Mucosa
/ cytology
Malabsorption Syndromes
/ genetics
Mice
Mutation
Nerve Tissue Proteins
/ chemistry
Organoids
/ cytology
Pancreas
/ cytology
Protein Binding
Protein Multimerization
Protein Stability
Neurogenin3
diabetes
digestive disease
disease mechanism
enteroendocrine
human organoids
intestine
pancreas
pluripotent stem cells
Journal
Developmental cell
ISSN: 1878-1551
Titre abrégé: Dev Cell
Pays: United States
ID NLM: 101120028
Informations de publication
Date de publication:
05 08 2019
05 08 2019
Historique:
received:
24
05
2018
revised:
25
02
2019
accepted:
06
05
2019
pubmed:
11
6
2019
medline:
7
3
2020
entrez:
11
6
2019
Statut:
ppublish
Résumé
Neurogenin3 (NEUROG3) is required for endocrine lineage formation of the pancreas and intestine. Patients with NEUROG3 mutations are born with congenital malabsorptive diarrhea due to complete loss of enteroendocrine cells, whereas endocrine pancreas development varies in an allele-specific manner. These findings suggest a context-dependent requirement for NEUROG3 in pancreas versus intestine. We utilized human tissue differentiated from NEUROG3
Identifiants
pubmed: 31178402
pii: S1534-5807(19)30389-2
doi: 10.1016/j.devcel.2019.05.017
pmc: PMC7082840
mid: NIHMS1529438
pii:
doi:
Substances chimiques
Basic Helix-Loop-Helix Transcription Factors
0
NEUROG3 protein, human
0
Nerve Tissue Proteins
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
367-380.e7Subventions
Organisme : NIDDK NIH HHS
ID : UG3 DK119982
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS044080
Pays : United States
Organisme : NICHD NIH HHS
ID : P01 HD093363
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK078392
Pays : United States
Organisme : NIGMS NIH HHS
ID : T32 GM063483
Pays : United States
Organisme : NIAID NIH HHS
ID : U19 AI116491
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA178974
Pays : United States
Organisme : NIDDK NIH HHS
ID : UH3 DK119982
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK092456
Pays : United States
Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.
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