Frimpter G W, Greenberg A J
J Clin Invest. 1967 Jun;46(6):975-82. doi: 10.1172/JCI105604.
Cystathionine is more readily cleared from the plasma than other amino acids. This is because the amino acid has a very low tubular maximum (Tm), in the order of 1 mumole per minute per 1.73 square meters body surface area (BSA). No essential differences in the reabsorption of cystathionine were observed in four normal subjects, two patients with homozygous cystathioninuria, one patient with heterozygous cystathioninuria, and one patient with cystinuria. Apparent net tubular secretion of cystathionine was demonstrated in a child with homozygous cystathioninuria only after an intravenously administered load of lysine.
同型半胱氨酸比其他氨基酸更容易从血浆中清除。这是因为该氨基酸的肾小管最大重吸收率(Tm)非常低,约为每分钟每1.73平方米体表面积(BSA)1微摩尔。在4名正常受试者、2名纯合子型同型半胱氨酸尿症患者、1名杂合子型同型半胱氨酸尿症患者和1名胱氨酸尿症患者中,未观察到同型半胱氨酸重吸收存在本质差异。仅在一名纯合子型同型半胱氨酸尿症儿童静脉注射赖氨酸负荷后,才证实存在同型半胱氨酸明显的肾小管净分泌。