G6PC1 and Decreased total neutrophil count: In neutrophils and macrophages, the G6PT/G6Pase-β complex preserves energy homeostasis and functionality, hence G6Pase-β mutations are responsible for GSD-Irs, an autosomal recessive disorder characterized by neutropenia and neutrophil dysfunction (Chou et al., 2010a,b), often associated with congenital cardiac and uro-genital anomalies (Boztug et al., 2009).