Tags: Disease.
Saccharopinuria (an excess of saccharopine in the urine) also called saccharopinemia saccharopine dehydrogenase deficiency or alpha-aminoadipic semialdehyde synthase deficiency is a variant form of hyperlysinemia. It is caused by a partial deficiency of the enzyme saccharopine dehydrogenase which plays a secondary role in the lysine metabolic pathway.
