Article
Shank3 mutation impairs glutamate signaling and myelination in ASD mouse model and human iPSC-derived OPCs.
Science advances - 11 Oct 2024
Fischer Inbar, Shohat Sophie, Leichtmann-Bardoogo Yael, Nayak Ritu, Wiener Gal, Rosh Idan, Shemen Aviram, Tripathi Utkarsh, Rokach May, Bar Ela, Hussein Yara, Castro Ana Carolina, Chen Gal, Soffer Adi, Schokoroy-Trangle Sari, Elad-Sfadia Galit, Assaf Yaniv, Schroeder Avi, Monteiro Patricia, Stern Shani, Maoz Ben M, Barak Boaz
Abstract excerpt
Autism spectrum disorder (ASD) is characterized by social and neurocognitive impairments, with mutations of the SHANK3 gene being prominent in patients with monogenic ASD. Using the InsG3680 mouse model with a Shank3 mutation seen in humans, we revealed an unknown role for Shank3 in postsynaptic oligodendrocyte (OL) features, similar to its role in neurons. This was shown by impaired molecular and physiological...
Topics
- Animals
- Induced Pluripotent Stem Cells
- Humans
- Nerve Tissue Proteins
- Mice
- Oligodendroglia
- Disease Models, Animal
- Myelin Sheath
- Glutamic Acid