Analysis of the role of sulfur amino acids metabolism in renal proximal tubules using mice lacking cysteine-producing enzymes

点击翻译按钮获取中文摘要

Cystathionine β-synthase (CBS) and cystathionine γ-lyase (CSE) are the transsulfuration enzymes that are involved in sulfur amino acids metabolism. CBS-deficient patients show severe clinical symptoms, while CSE-deficient persons are considered to be free of any clinical manifestations. Although both enzymes are primarily expressed in the liver and the kidney of mice, their roles in the kidney remain to be elucidated. In CBS-deficient mice, the urinary excretion of toxic methionine was much lower efficiency, while the cystathionine was efficiently excreted into the urine in CSE-deficient mice. The efficiency of excretion is considered to be related to the difference of symptoms. Pregnancy-induced hypertension and proteinuria was developed in normal-appearing CSE-deficient mice. H2S that has vasorelaxant activity is known to be produced by CBS and CSE, and its distribution was overlapped with both enzymes in kidney.