Meeting Banner
Abstract #4516

Monitoring muscle glycogen accumulation in the GAAKO mouse model of Pompe disease with glycogen CEST (GlyCEST) MRI

Limin Wu1, Hahnsung Kim2, Julius Chung2, and Phillip Sun2
1Neuroscience Center and Department of Pediatrics,MGH, Charlestown, MA, United States, 2Emory University, Atlanta, GA, United States

Synopsis

Keywords: Muscle, MSK

Motivation: Pompe disease is an autosomal recessive metabolic disorder that is characterized by a deficiency of the lysosomal enzyme alpha-glucosidase (GAA), resulting in massive glycogen deposition in muscle. It helps to develop advanced CEST MRI to help manage Pompe disease.

Goal(s): We aimed to develop and test quantitative glycogen CEST (GlyCEST) MRI for non-invasive monitoring of disease progression in GAAKO mice.

Approach: We performed longitudinal GlyCEST scans in wild-type and GAAKO mice and tested the correlation between QUASS-boosted quantitative glycogen MRI with glycogen assay.

Results: GlyCEST MRI provides a surrogate MRI biomarker that significantly correlates with the muscle glycogen biochemical assay.

Impact: GlyCEST is promising to monitor glycogen levels in Pompe mice in real-time non-invasively and is potentially valuable for monitoring treatment response and ultimately clinical translation.

How to access this content:

For one year after publication, abstracts and videos are only open to registrants of this annual meeting. Registrants should use their existing login information. Non-registrant access can be purchased via the ISMRM E-Library.

After one year, current ISMRM & ISMRT members get free access to both the abstracts and videos. Non-members and non-registrants must purchase access via the ISMRM E-Library.

After two years, the meeting proceedings (abstracts) are opened to the public and require no login information. Videos remain behind password for access by members, registrants and E-Library customers.

Click here for more information on becoming a member.

Keywords