The cellular GSH concentration in the cells sorted as GSH-high hES-MSCs was significantly higher than that of cells sorted into the GSH-low group (Fig
The affected infant may exhibit dysmorphic features, including microcephaly, overfolded helices, a sloping forehead, long and tapered fingers and toes, contractures, and hypoplastic toenails ( Among the various phenotypic forms of CPT II deficiency, the severe infantile hepatocardiomuscular type typically presents with hypoketotic hypoglycemia, liver failure, and cardiomyopathy within the first year of life
With regard to catalytic mechanism, all the seleno-GPxs share the common pathway which starts with the binding of hydroperoxides and GSH at the catalytic and thiol binding motifs, respectively [8, 11,12,13,14,15,16,17,18]
Their role in specific areas, such as fat metabolism and weight loss, remains unclear and is not substantiated by significant clinical evidence