Alexander Disease Modeling in Zebrafish: An In Vivo System Suitable to Perform Drug Screening.
Alexander disease
glial fibrillary acid protein
microinjection
zebrafish
Journal
Genes
ISSN: 2073-4425
Titre abrégé: Genes (Basel)
Pays: Switzerland
ID NLM: 101551097
Informations de publication
Date de publication:
11 12 2020
11 12 2020
Historique:
received:
13
11
2020
revised:
04
12
2020
accepted:
07
12
2020
entrez:
16
12
2020
pubmed:
17
12
2020
medline:
23
7
2021
Statut:
epublish
Résumé
Alexander disease (AxD) is a rare astrogliopathy caused by heterozygous mutations, either inherited or arising de novo, on the glial fibrillary acid protein (GFAP) gene (17q21). Mutations in the GFAP gene make the protein prone to forming aggregates which, together with heat-shock protein 27 (HSP27), αB-crystallin, ubiquitin, and proteasome, contribute to form Rosenthal fibers causing a toxic effect on the cell. Unfortunately, no pharmacological treatment is available yet, except for symptom reduction therapies, and patients undergo a progressive worsening of the disease. The aim of this study was the production of a zebrafish model for AxD, to have a system suitable for drug screening more complex than cell cultures. To this aim, embryos expressing the human
Identifiants
pubmed: 33322348
pii: genes11121490
doi: 10.3390/genes11121490
pmc: PMC7764705
pii:
doi:
Substances chimiques
GFAP protein, human
0
Glial Fibrillary Acidic Protein
0
Ceftriaxone
75J73V1629
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
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