FBP1 and hyper-beta-alaninemia: As a rate-limiting enzyme, FBP1 catalyzes the reaction of fructose 1,6-bisphosphate to fructose 6-phosphate, while the deficiency of FBP1 increases the levels of uric acid and hyperalaninemia with the consequence of metabolic acidosis or ketosis (Bijarnia-Mahay et al., 1993).