In reverse, ketoacidosis is a typical finding in organic acidurias (such as maple syrup urine disease, propionic aciduria, methylmalonic aciduria, isovaleric aciduria), gluconeogenesis defects (such as fructose-1, 6-bisphosphatase, pyruvatecarboxylase, phosphoenolpyruvate carboxykinase deficiencies), glycogenolysis, ketolysis and respiratory chain defects (Table 2). This evidence concerns the gene FBP1 and maple syrup urine disease.