Similä S
Ann Clin Biochem. 1979 Jul;16(4):177-81. doi: 10.1177/000456327901600143.
Hydroxyproline metabolism was studied in two patients with type II hyperprolinaemia (HP II) using oral loadings of hydroxyproline or hydroxyproline-ornithine. delta 1-pyrroline-3-hydroxy-5-carboxylic acid (3 OH-PC) and delta 1-pyrroline-5-carboxylic acid (PC) were identified in the urine. The urinary excretion of both 3-OH-PC and PC increased in HP II patients but not in healthy controls during oral loading of hydroxyproline and hydroxyproline-ornithine. The plasma level of proline in patients with HP II is very high but the hydroxyproline concentration is normal or only slightly increased. Therefore one can assume that hydroxyproline is converted to pyrrole-2-carboxylic acid, which is excreted in urine as a glycine conjugate. In this study it was demonstrated that the highly elevated plasma level of proline in one of the patients with HP II decreased greatly after hydroxyproline-ornithine load; this change was followed by a 40-fold rise in urinary excretion of proline.
采用口服羟脯氨酸或羟脯氨酸 - 鸟氨酸负荷试验，对两名II型高脯氨酸血症（HP II）患者的羟脯氨酸代谢进行了研究。在尿液中鉴定出了δ1 - 吡咯啉 - 3 - 羟基 - 5 - 羧酸（3 - OH - PC）和δ1 - 吡咯啉 - 5 - 羧酸（PC）。在口服羟脯氨酸和羟脯氨酸 - 鸟氨酸负荷试验期间，HP II患者尿液中3 - OH - PC和PC的排泄量均增加，而健康对照者则未增加。HP II患者的血浆脯氨酸水平非常高，但羟脯氨酸浓度正常或仅略有升高。因此可以推测，羟脯氨酸被转化为吡咯 - 2 - 羧酸，并以甘氨酸共轭物的形式随尿液排出。在本研究中，证明了其中一名HP II患者在口服羟脯氨酸 - 鸟氨酸负荷后，血浆中高度升高的脯氨酸水平大幅下降；随后，尿液中脯氨酸的排泄量增加了40倍。
