Chamson A, Voigtländer V, Myara I, Frey J
Biochemistry Department, Medical School, Saint-Etienne, France.
Clin Physiol Biochem. 1989;7(3-4):128-36.
Prolidase deficiency is a rare hereditary disease characterized by an iminodipeptiduria especially composed by glycyl-L-proline which is not further degraded. The study of collagen metabolism in fibroblast cultures from three prolidase-deficient patients showed an increase in the rapidly degraded collagen and a decrease in the proline pool. In order to elucidate the mechanism of this metabolic disturbance, glycyl-L-proline was added to the cell cultures. In the control cultures, the addition of this dipeptide caused an increase in the rapidly degraded collagen and a decrease in the proline pool. The effects on the patient fibroblasts depended on the severity of the deficiency. The metabolic function of the dipeptide glycyl-L-proline was discussed in the light of these results.
脯氨酰肽酶缺乏症是一种罕见的遗传性疾病，其特征为亚氨基二肽尿症，尤其是由未进一步降解的甘氨酰-L-脯氨酸组成。对三名脯氨酰肽酶缺乏症患者的成纤维细胞培养物中的胶原蛋白代谢进行研究发现，快速降解的胶原蛋白增加，脯氨酸池减少。为了阐明这种代谢紊乱的机制，将甘氨酰-L-脯氨酸添加到细胞培养物中。在对照培养物中，添加这种二肽导致快速降解的胶原蛋白增加，脯氨酸池减少。对患者成纤维细胞的影响取决于缺乏的严重程度。根据这些结果讨论了二肽甘氨酰-L-脯氨酸的代谢功能。
