Abstract #2594

MRI monitors the abnormal muscle glycogen and creatine kinase activity in Pompe disease mice

Shaowei Bo^1,2, Chongxue Bie^1,3, Nirbhay N. Yadav^4,5, Peter C.M. van Zijl^4,5, Jiadi Xu^4,5, Chao Zou¹, Hairong Zheng¹, and Yang Zhou¹

¹Shenzhen Institute of Advanced Technology, Chinese Academy of Sciences, Shenzhen, China, ²Medical Imaging Center, The First Affiliated Hospital of Jinan University, Guangzhou, China, ³Department of Information Science and Technology, Northwest University, Xian, China, ⁴F.M Kirby Research Center, Kennedy Krieger Institute, Baltimore, MD, United States, ⁵Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, United States

Synopsis

Keywords: Muscle, CEST & MT

Pompe disease (PD) is a glycogen storage disease, characterized by progressive lysosomal glycogen accumulation and changes in energy metabolite levels. There is a lack of methods to noninvasively assess PD progression and treatment response. We demonstrate that saturation transfer MRI can detect changes in glycogen, total creatine (tCr), and phosphocreatine (PCr) in a mouse model of PD and show more than doubling of muscle glycogen levels and a gradual decrease of tCr and PCr levels with age. The simultaneous mapping of these energy metabolites with MRI has potential to assess PD and other diseases involving mitochondrial or energy metabolic disorders.

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