The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500. Detects a band of approximately 84 kDa (predicted molecular weight: 84 kDa). Incubate membrane with diluted antibody in 5% nonfat milk, 1X TBS, 0.1% Tween-20 at 4°C with gentle shaking over night.
FunctionTransfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan.
Involvement in diseaseDefects in GYS1 are the cause of muscle glycogen storage disease type 0 (GSD0b) [MIM:611556]; also known as muscle glycogen synthase deficiency. GSD0b is a metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood. The role of muscle glycogen is to provide critical energy during bursts of activity and sustained muscle work.
Sequence similaritiesBelongs to the glycosyltransferase 3 family.