The top five highly significant pathways affected by both mouse Cbs deficiency and human CBS deficiency were Acute Phase Response Signaling (− log[p-value] = 25.9 [humans] and 33.5 [mice]), LXR/RXR Activation (− log[p-value] = 30.8 [humans] and 31.4 [mice]), FXR/RXR Activation (− log[p-value] = 30.5 [humans] and 30.0 [mice]), Coagulation System, and Complement System (Fig. 4B). This evidence concerns the gene NR1H4 and hyperinsulinemic hypoglycemia, familial, 4.