Human embryoid bodies as a 3D tissue model of the extracellular matrix and α-dystroglycanopathies.
Basement Membrane
/ drug effects
Case-Control Studies
Cell Culture Techniques
Cells, Cultured
Child
Child, Preschool
Dystroglycans
/ genetics
Embryoid Bodies
/ drug effects
Extracellular Matrix
/ drug effects
Female
Gene Expression Regulation, Developmental
Human Embryonic Stem Cells
/ drug effects
Humans
Induced Pluripotent Stem Cells
/ drug effects
Infant, Newborn
Male
Middle Aged
Ribitol
/ pharmacology
Walker-Warburg Syndrome
/ drug therapy
Dystroglycan
Extracellular matrix
Muscular dystrophy
Stem cells
Journal
Disease models & mechanisms
ISSN: 1754-8411
Titre abrégé: Dis Model Mech
Pays: England
ID NLM: 101483332
Informations de publication
Date de publication:
26 06 2020
26 06 2020
Historique:
received:
17
10
2019
accepted:
30
04
2020
pubmed:
20
5
2020
medline:
24
8
2021
entrez:
20
5
2020
Statut:
epublish
Résumé
The basal lamina is a specialized sheet of dense extracellular matrix (ECM) linked to the plasma membrane of specific cell types in their tissue context, which serves as a structural scaffold for organ genesis and maintenance. Disruption of the basal lamina and its functions is central to many disease processes, including cancer metastasis, kidney disease, eye disease, muscular dystrophies and specific types of brain malformation. The latter three pathologies occur in the α-dystroglycanopathies, which are caused by dysfunction of the ECM receptor α-dystroglycan. However, opportunities to study the basal lamina in various human disease tissues are restricted owing to its limited accessibility. Here, we report the generation of embryoid bodies from human induced pluripotent stem cells that model the basal lamina. Embryoid bodies cultured via this protocol mimic pre-gastrulation embryonic development, consisting of an epithelial core surrounded by a basal lamina and a peripheral layer of ECM-secreting endoderm. In α-dystroglycanopathy patient embryoid bodies, electron and fluorescence microscopy reveal ultrastructural basal lamina defects and reduced ECM accumulation. By starting from patient-derived cells, these results establish a method for the
Identifiants
pubmed: 32423971
pii: dmm.042986
doi: 10.1242/dmm.042986
pmc: PMC7328151
pii:
doi:
Substances chimiques
Dystroglycans
146888-27-9
Ribitol
488-81-3
Types de publication
Journal Article
Research Support, N.I.H., Intramural
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2020. Published by The Company of Biologists Ltd.
Déclaration de conflit d'intérêts
Competing interestsThe authors declare no competing or financial interests.
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