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1
Renal clearance of cystathionine in homozygous and heterozygous cystathioninuria, cystinuria, and the normal state.同型胱氨酸尿症纯合子和杂合子、胱氨酸尿症以及正常状态下胱硫醚的肾清除率。
J Clin Invest. 1967 Jun;46(6):975-82. doi: 10.1172/JCI105604.
2
Disorders of amino acid transport.氨基酸转运障碍
Biochem Soc Trans. 1983 Dec;11(6):633-4. doi: 10.1042/bst0110633.
3
Disorders of intestinal transport of amino acids.
Am J Dis Child. 1969 Jan;117(1):13-23. doi: 10.1001/archpedi.1969.02100030015003.
4
Renal handling of dibasic amino acids and cystine in cystinuria.胱氨酸尿症中二元氨基酸和胱氨酸的肾脏处理
Clin Sci Mol Med. 1977 Jul;53(1):9-15. doi: 10.1042/cs0530009.
5
[The inborn errors of metabolism of amino acids].[氨基酸代谢的先天性缺陷]
Postepy Biochem. 1973;19(1):91-122.
6
Renal transport of lysine and arginine in cystinuria.胱氨酸尿症中赖氨酸和精氨酸的肾脏转运
Tohoku J Exp Med. 1983 Jan;139(1):9-16. doi: 10.1620/tjem.139.9.
7
DISORDERS OF AMINO ACID TRANSPORT.氨基酸转运障碍
Ann Intern Med. 1965 Apr;62:847-51. doi: 10.7326/0003-4819-62-4-847.
8
[Defects of renal tubular transport systems for amino acids].[肾小管氨基酸转运系统的缺陷]
Med Klin. 1975 Feb 21;70(8):301-12.
9
Defective metabolic clearance of plasma arginine and ornithine in lysinuric protein intolerance.赖氨酸尿性蛋白不耐受症中血浆精氨酸和鸟氨酸的代谢清除缺陷。
Metabolism. 1974 Aug;23(8):691-701. doi: 10.1016/0026-0495(74)90001-8.
10
[Amino acid transfer systems and their importance in pathology. II. Specific abnormalities in renal and intestinal amino acid transfer].
Ann Biol Clin (Paris). 1973;31(4):249-55.

引用本文的文献

1
Studies on cystathionase activity in rat liver and brain during development. Effects of hormones and amino acids in vivo.发育过程中大鼠肝脏和大脑中胱硫醚酶活性的研究。体内激素和氨基酸的影响。
Biochem J. 1973 Dec;136(4):1011-5. doi: 10.1042/bj1361011.

本文引用的文献

1
Determination of inulin by means of resorcinol.用间苯二酚法测定菊粉。
Proc Soc Exp Biol Med. 1950 May;74(1):117-20. doi: 10.3181/00379727-74-17827.
2
Aminoacid metabolism in cystinuria.胱氨酸尿症中的氨基酸代谢
Q J Med. 1951 Jul;20(79):205-19.
3
Cystathioniuria.胱硫醚尿症
Ann Hum Genet. 1959 Dec;23:442-53. doi: 10.1111/j.1469-1809.1959.tb01485.x.
4
HOMOCYSTINURIA. BIOCHEMICAL STUDIES OF TISSUES INCLUDING A COMPARISON WITH CYSTATHIONINURIA.同型胱氨酸尿症。组织的生化研究,包括与胱硫醚尿症的比较。
Pediatrics. 1965 Jan;35:50-6.
5
CYSTINURIA: METABOLISM OF THE DISULFIDE OF CYSTEINE AND HOMOCYSTEINE.胱氨酸尿症:半胱氨酸和高半胱氨酸二硫化物的代谢
J Clin Invest. 1963 Dec;42(12):1956-64. doi: 10.1172/JCI104882.
6
THE RENAL EXCRETION OF FOLIC ACID.叶酸的肾脏排泄
J Clin Invest. 1963 Dec;42(12):1841-9. doi: 10.1172/JCI104868.
7
STUDIES OF FUNCTIONAL NEURAL TUMORS. II. CYSTATHIONINURIA.
Scand J Clin Lab Invest. 1963;15:474-8.
8
Effect of penicillamine on cystinuria.青霉胺对胱氨酸尿症的影响。
Br Med J. 1963 Mar 2;1(5330):588-90. doi: 10.1136/bmj.1.5330.588.
9
Aminoacid metabolism in cystinuria.胱氨酸尿症中的氨基酸代谢
Clin Sci. 1962 Oct;23:285-304.
10
Cystathioninuria.
N Engl J Med. 1963 Feb 14;268:333-9. doi: 10.1056/NEJM196302142680701.

同型胱氨酸尿症纯合子和杂合子、胱氨酸尿症以及正常状态下胱硫醚的肾清除率。

Renal clearance of cystathionine in homozygous and heterozygous cystathioninuria, cystinuria, and the normal state.

作者信息

Frimpter G W, Greenberg A J

出版信息

J Clin Invest. 1967 Jun;46(6):975-82. doi: 10.1172/JCI105604.

DOI:10.1172/JCI105604
PMID:6026103
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC297102/
Abstract

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.

摘要

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。