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5-Oxoprolinase deficiency is a very rare autosomal recessive disease characterized by 5-oxoprolinuria, and a very heterogeneous clinical presentation, including renal stone formation, enterocolitis, mental retardation, neonatal hypoglycemia, microcytic anemia and microcephaly (Njalsson and Norgren, 2005

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It is likely that lipidation occurs in tissues or organelles where proteins have a slow turnover, but this is challenging to prove