<unk>  (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. Inheritance is thought to be autosomal recessive, but this cannot be established as individuals affected by<unk>  typically have only a 40% reduction in functional enzyme.
Saccharopinuria
Urocanic aciduria
Isosthenuria
Hawkinsinuria
Climacturia
Pentosuria
Sucrose intolerance
Orotic aciduria