Lauren M Rosko, Tyler Gentile, Victoria N Smith, Zeeba Manavi, George S Melchor, Jingwen Hu, Nataliia V Shults, Chris Albanese, Yichien Lee, Olga Rodriguez, Jeffrey K Huang
Cerebral creatine deficiency syndrome (CCDS) is an inborn error of metabolism characterized by intellectual delays, seizures, and autistic-like behavior. However, the role of endogenously synthesized creatine on central nervous system (CNS) development and function remains poorly understood. Here, magnetic resonance spectroscopy (MRS) of adult mouse brains from both sexes revealed creatine synthesis is dependent on the expression of the enzyme, guanidinoacetate methyltransferase (GAMT). To identify Gamt -expressed cells, and how Gamt affects postnatal CNS development, we generated a mouse line by knocking-in a green fluorescent protein (GFP) which is expressed upon excision of Gamt We found that Gamt is expressed in mature oligodendrocytes during active myelination in the developing postnatal CNS...
January 27, 2023: Journal of Neuroscience