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hyperprolinemia 1. an autosomal recessive aminoacidopathy characterized by excessive proline in the body fluids; it occurs as two types, both of which are probably benign. Type I is caused by deficiency of proline oxidase; type II is due to deficiency of 1-pyrroline-5-carboxylate dehydrogenase and is characterized by accumulation of higher levels of proline and by urinary excretion of Δ1-pyrroline 5-carboxylate.  2. excess of proline in the blood; called also prolinemia.
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