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- Title
Dysregulation of astrocyte extracellular signaling in Costello syndrome.
- Authors
Krencik, Robert; Hokanson, Kenton C.; Narayan, Aditi R.; Dvornik, Jill; Rooney, Gemma E.; Rauen, Katherine A.; Weiss, Lauren A.; Rowitch, David H.; Ullian, Erik M.
- Abstract
The article focuses on astroglial lineage was investigated in model systems of a developmental disorder with intellectual disability caused by mutant Harvey rat sarcoma viral oncogene homolog (HRAS) termed Costello syndrome. It mentions HRAS human induced pluripotent stem cells (iPSCs) and transgenic mice and farnesyl transferase inhibitor and knockdown of transcription factor SNAI2. It also mentions perineuronal net proteoglycans as well as increased parvalbumin expression in interneurons.
- Subjects
INTELLECTUAL disabilities; HRAS genes; COSTELLO syndrome; INDUCED pluripotent stem cells; TRANSGENIC mice; FARNESYLTRANSFERASE; TRANSCRIPTION factors; PARVALBUMINS
- Publication
Science Translational Medicine, 2015, Vol 7, Issue 286, p1
- ISSN
1946-6234
- Publication type
Article
- DOI
10.1126/scitranslmed.aaa5645