BACKGROUND Hyperprolinemia is a rare inherited metabolic disorder characterized by a high proline level in blood and/or urine and various neuropsychiatric symptoms. Type I hyperprolinemia is caused by a proline oxidase deficiency, which is encoded by the PRODH gene on chromosome 22q11. Herein, we present a study of Korean patients with type I hyperprolinemia who were diagnosed during newborn sc...

The metabolism of the nonessential amino acid proline contributes to tumor metabolic reprogramming. Previously we showed that MYC increases proline biosynthesis (PB) from glutamine. Here we show MYC increases the expression of the enzymes in PB at both protein and mRNA levels. Blockade of PB decreases tumor cell growth and energy production. Addition of Δ(1)-pyrroline-5-carboxylate (P5C) or pro...

Purpose: This study aimed to assess the effects of sowing date and nitrogen application rate on grain yield, use efficiency (NUE), 2-acetyl-△1-pyrroline (2-AP) contents biochemical parameters related 2-AP formation in fragrant rice. Methods: A factorial split-plot arrangement treatments was set up a split-zone experiment with two factors three levels carried out for 3 years. The main plots incl...

In transgenic Arabidopsis a patatin class I promoter from potato is regulated by sugars and proline (Pro), thus integrating signals derived from carbon and nitrogen metabolism. In both cases a signaling cascade involving protein phosphatases is involved in induction. Other endogenous genes are also regulated by both Pro and carbohydrates. Chalcone synthase (CHS) gene expression is induced by bo...

PRODH, encoding proline oxidase (POX), has been associated with schizophrenia through linkage, association, and the 22q11 deletion syndrome (Velo-Cardio-Facial syndrome). Here, we show in a family-based sample that functional polymorphisms in PRODH are associated with schizophrenia, with protective and risk alleles having opposite effects on POX activity. Using a multimodal imaging genetics app...

The effect of various NaCl treatments (0, 50, 100, 200 and 300 mM) at different growth and development stages (tillering, boot swelling, flowering and polination) of two wheat cultivars (Ghods : salt-sensitive; Boolani : salt-resistant) on proline concentration and the kinetic activity of proline dehydrogenase was studied under greenhouse conditions. Generally, in response to salinity treatment...

Degradation of the multifunctional amino acid proline is associated with mitochondrial oxidative respiration. The two-step oxidation of proline is catalyzed by proline oxidase and Δ(1)-pyrroline-5-carboxylate (P5C) dehydrogenase, which produce P5C and glutamate, respectively. In animal and plant cells, impairment of P5C dehydrogenase activity results in P5C-proline cycling when exogenous prolin...

Despite decades of research being conducted to understand what physiological deficits in the brain are an underlying basis psychiatric diseases like schizophrenia, it has remained difficult establish a direct causal relationship between neuronal dysfunction and specific behavioral phenotypes. Moreover, remains unclear how metabolic processes, including amino acid metabolism, affect function con...