Dystrophin deficiency leads to dysfunctional glutamate clearance in iPSC derived astrocytes

• Published in: Translational psychiatry Vol. 9; no. 1; pp. 200 - 21
• Main Authors: Patel, Abdulsamie M., Wierda, Keimpe, Thorrez, Lieven, van Putten, Maaike, De Smedt, Jonathan, Ribeiro, Luis, Tricot, Tine, Gajjar, Madhavsai, Duelen, Robin, Van Damme, Philip, De Waele, Liesbeth, Goemans, Nathalie, Tanganyika-de Winter, Christa, Costamagna, Domiziana, Aartsma-Rus, Annemieke, van Duyvenvoorde, Hermine, Sampaolesi, Maurilio, Buyse, Gunnar M., Verfaillie, Catherine M.
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 21.08.2019; Nature Publishing Group
• Subjects: 13/100; 13/106; 13/31; 14/19; 14/56; 38/39; 38/77; 631/532; 692/699/476/1373; Astrocytes - cytology; Astrocytes - metabolism; Behavioral Sciences; Biological Psychology; Calcium - metabolism; Cytoskeleton - metabolism; Defects; Dystrophin - genetics; Dystrophin - metabolism; Glutamic Acid - metabolism; Homeostasis; Humans; Induced Pluripotent Stem Cells - cytology; Induced Pluripotent Stem Cells - metabolism; Male; Medicine; Medicine & Public Health; Muscular Dystrophy, Duchenne - genetics; Muscular Dystrophy, Duchenne - metabolism; Mutation; Neuronal Outgrowth - physiology; Neurons - cytology; Neurons - metabolism; Neurosciences; Nitric Oxide - metabolism; Pharmacotherapy; Psychiatry

Duchenne muscular dystrophy (DMD) results, beside muscle degeneration in cognitive defects. As neuronal function is supported by astrocytes, which express dystrophin, we hypothesized that loss of dystrophin from DMD astrocytes might contribute to these cognitive defects. We generated cortical neuron...