Mutations in GFAP Alter Early Lineage Commitment of Organoids
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F68378041%3A_____%2F25%3A00638093" target="_blank" >RIV/68378041:_____/25:00638093 - isvavai.cz</a>
Alternative codes found
RIV/86652036:_____/25:00638351 RIV/00216208:11310/25:10515586
Result on the web
<a href="https://onlinelibrary.wiley.com/doi/10.1002/glia.70049" target="_blank" >https://onlinelibrary.wiley.com/doi/10.1002/glia.70049</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1002/glia.70049" target="_blank" >10.1002/glia.70049</a>
Alternative languages
Result language
angličtina
Original language name
Mutations in GFAP Alter Early Lineage Commitment of Organoids
Original language description
Glial fibrillary acidic protein (GFAP) is a type-3 intermediate filament protein mainly expressed in astrocytes in the central nervous system. Mutations in GFAP cause Alexander disease (AxD), a rare and fatal neurological disorder. How exactly mutant GFAP eventually leads to white and gray matter deterioration in AxD remains unknown. GFAP is known to be expressed also in neural precursor cells in the developing brain. Here, we used AxD patient-derived induced pluripotent stem cells (iPSCs) to explore the impact of mutant GFAP during neurodifferentiation. Our results show that GFAP is already expressed in iPSCs. Moreover, we have found that mutations in GFAP can severely affect neural organoid development through altering lineage commitment in embryoid bodies. Together, these results support the notion that GFAP plays a role as an early modulator of neurodevelopment.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30103 - Neurosciences (including psychophysiology)
Result continuities
Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2025
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Glia
ISSN
0894-1491
e-ISSN
1098-1136
Volume of the periodical
73
Issue of the periodical within the volume
11
Country of publishing house
US - UNITED STATES
Number of pages
22
Pages from-to
2167-2188
UT code for WoS article
001540289700001
EID of the result in the Scopus database
2-s2.0-105011937143