A Modified Enzymatic Method for Measurement of Glycogen Content in Glycogen Storage Disease Type IV

Abstract

Deficiency of glycogen branching enzyme in glycogen storage disease type IV (GSD IV) results in accumulation of less-branched and poorly soluble polysaccharides (polyglucosan bodies) in multiple tissues.

Systemic Correction of Murine Glycogen Storage Disease Type IV by an AAV-Mediated Gene Therapy

“…Color images available online at www.liebertpub.com/hum measurement. 30 At 3 months of age, glycogen contents in the skeletal muscles (quadriceps and gastrocnemius) of AAV-treated mice were at similar levels of Wt, indicating an effective correction of glycogen storage in skeletal muscles. The glycogen content in the AAV-treated mice was 59% in the fasting liver, and 48% in the brain of those in UT mice (Fig.…”

“…Recently, a modified method was developed that includes an extra boiling step to dissolve the insoluble glycogen, ultimately preserving the glycogen in tissue homogenates from Gbe1 ys/ys mice. 30 Blood chemistry tests could provide important clinical information of disease progression and treatment outcome. Elevation of serum aminotransferases AST and ALT is commonly seen in patients with GSD IV, and fasting hypoglycemia is present in some patients with liver cirrhosis.…”

Alglucosidase alfa treatment alleviates liver disease in a mouse model of glycogen storage disease type IV

“…Tissue GAA activity was analyzed as described [15] . Glycogen content was measured using a modified method that is suitable for GSD IV tissues [16] . Protein concentration was measured using BCA method.…”

Knockout of murine Lyplal1 confers sex-specific protection against diet-induced obesity

“…The copyright holder for this preprint this version posted August 5, 2021. ; https://doi.org/10.1101/2021.08.05.455257 doi: bioRxiv preprint triglyceride kit (Sigma, St. Louis, MO, USA) and absorbance at nm measured using an Eppendorf Biospectrometer. D. Glycogen extraction: Glycogen was extracted from frozen liver tissue samples based on a modified version of a previously published protocol 56 . In brief, 0.05 g of liver tissue was homogenized on ice in 4ml of distilled water using a Benchmark D1000 Handheld Homogenizer (Benchmark Scientific, Edison, NJ, USA).…”