赖氨酸尿性蛋白不耐受症中赖氨酸的基底外侧膜转运缺陷。

Basolateral-membrane transport defect for lysine in lysinuric protein intolerance.

作者信息

Rajantie J, Simell O, Perheentupa J

出版信息

Lancet. 1980 Jun 7;1(8180):1219-21. doi: 10.1016/s0140-6736(80)91679-7.

Abstract

In lysinuric protein intolerance intestinal, hepatic, and renal diaminoacid transport is defective, causing malnutrition. In patients fed with lysylglycine plasma glycine increased normally, but lysine increased as poorly as with free lysine. This is the first demonstration of defective peptide absorption. The primary defect is suggested to be at the basolateral cell membrane.

摘要

在赖氨酸尿性蛋白不耐受症中，肠道、肝脏和肾脏的二氨基酸转运存在缺陷，导致营养不良。在喂食赖氨酰甘氨酸的患者中，血浆甘氨酸正常增加，但赖氨酸的增加与游离赖氨酸一样少。这是肽吸收缺陷的首次证明。主要缺陷被认为位于基底外侧细胞膜。