Mutations in GFAP Alter Early Lineage Commitment of Organoids

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/86652036:_____/25:00638351 RIV/00216208:11310/25:10515586

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Mutations in GFAP Alter Early Lineage Commitment of Organoids

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Mutations in GFAP Alter Early Lineage Commitment of Organoids

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30103 - Neurosciences (including psychophysiology)

Projekt

Výsledek vznikl pri realizaci vícero projektů. Více informací v záložce Projekty.

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2025

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Glia

ISSN

0894-1491

e-ISSN

1098-1136

Svazek periodika

73

Číslo periodika v rámci svazku

11

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

22

Strana od-do

2167-2188

Kód UT WoS článku

001540289700001

EID výsledku v databázi Scopus

2-s2.0-105011937143