Impaired myogenic development, differentiation and function in hESC-derived SMA myoblasts and myotubes
Fig 6
Impaired glycolytic function and metabolic activity in myoblasts from SMA patients.
Analysis of glycolysis with the glycolysis rate assay using the Seahorse revealed decreased levels of compensatory glycolysis in SMA Type 1 myoblasts (A, B). Mitochondrial stress test detected impaired oxidative phosphorylation in SMA Type 1 myoblasts ranging from reduced levels during basal respiration, ATP production, maximal respiration to spare capacity and proton leak (C, D). ECAR: extracellular acidification rate, OCR: oxidative consumption rate, Data represented as mean ± SEM of n = 3 individual samples. *: p < 0:05, **: p < 0:01, ***: p < 0:001, two-tailed unpaired Student's t-test.