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Renal transport of aromatic acids in patients with phenylketonuria.

作者信息

Langenbeck U, Behbehani A, Luthe H

出版信息

J Inherit Metab Dis. 1981;4(2):69-70. doi: 10.1007/BF02263595.

DOI:10.1007/BF02263595
PMID:6790852
Abstract

Renal clearance of phenylpyruvic acid is maximal at a plasma concentration of 40-60 mumol/l. This concentration is obtained with plasma phenylalanine concentrations of 1.0-1.2 mmol/l, the threshold for separating classical phenylketonuria from phenylketonuria variants.

摘要

相似文献

1
Renal transport of aromatic acids in patients with phenylketonuria.
J Inherit Metab Dis. 1981;4(2):69-70. doi: 10.1007/BF02263595.
2
Phenylketonemia in phenylketonuria.
Neuropadiatrie. 1974 May;5(2):125-37. doi: 10.1055/s-0028-1091695.
3
A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.苯丙酮尿症中苯丙氨酸非共轭酸性转氨代谢产物的综述。
J Inherit Metab Dis. 1992;15(1):136-44. doi: 10.1007/BF01800355.
4
Clearance of endogenous phenylpyruvate in phenylketonurics.苯丙酮尿症患者体内内源性苯丙酮酸的清除
Clin Chim Acta. 1969 Jul;25(1):179-80. doi: 10.1016/0009-8981(69)90247-2.
5
Absence of a significant renal threshold for two aromatic acids in phenylketonuric children over two years of age.
Eur J Pediatr. 1980 Aug;134(2):115-8. doi: 10.1007/BF01846027.
6
Aromatic acid excretion in classical phenylketonuria and hyperphenylalaninemic variants.经典型苯丙酮尿症和高苯丙氨酸血症变异型中的芳香酸排泄
Helv Paediatr Acta. 1974 Nov;29(5):489-98.
7
Tryptophan oxidation in phenylketonuria.苯丙酮尿症中的色氨酸氧化
Pediatr Res. 1967 Sep;1(5):372-85. doi: 10.1203/00006450-196709000-00005.
8
Problems of routine screening for phenylketonuria.
Lancet. 1962 Sep 8;2(7254):498-500. doi: 10.1016/s0140-6736(62)90358-6.
9
[Further problems and perspectives of phenylketonuria. Phenylketonuria and phenylalaninemia].[苯丙酮尿症的进一步问题与前景。苯丙酮尿症与苯丙氨酸血症]
Presse Med (1893). 1966 Nov 19;74(49):2541-6.
10
Quantitative studies on the urinary excretion of unconjugated aromatic acids in phenylketonuria.苯丙酮尿症中未结合芳香酸尿排泄的定量研究。
Clin Chim Acta. 1974 Sep 30;55(3):281-94. doi: 10.1016/0009-8981(74)90002-3.

引用本文的文献

1
Carriership of the rs113883650/rs2287120 haplotype of the () gene increases the risk of obesity in infants with phenylketonuria.()基因的rs113883650/rs2287120单倍型携带增加了苯丙酮尿症婴儿肥胖的风险。 (注:原文括号处信息缺失)
Mol Genet Metab Rep. 2020 Aug 21;25:100640. doi: 10.1016/j.ymgmr.2020.100640. eCollection 2020 Dec.
2
A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria.苯丙酮尿症中苯丙氨酸非共轭酸性转氨代谢产物的综述。
J Inherit Metab Dis. 1992;15(1):136-44. doi: 10.1007/BF01800355.

本文引用的文献

1
The renal clearance of phenylpyruvate.
Clin Chim Acta. 1961 Nov;6:813-8. doi: 10.1016/0009-8981(61)90170-x.
2
Absence of a significant renal threshold for two aromatic acids in phenylketonuric children over two years of age.
Eur J Pediatr. 1980 Aug;134(2):115-8. doi: 10.1007/BF01846027.
3
Clearance of endogenous phenylpyruvate in phenylketonurics.苯丙酮尿症患者体内内源性苯丙酮酸的清除
Clin Chim Acta. 1969 Jul;25(1):179-80. doi: 10.1016/0009-8981(69)90247-2.
4
Mental retardation and inborn errors of metabolism.
智力迟钝与先天性代谢缺陷
Lancet. 1973 Apr 7;1(7806):785. doi: 10.1016/s0140-6736(73)92190-9.
5
Quantitative analysis of beta-phenylpyruvic acid by single ion monitoring. Evaluation of isomeric internal standards.通过单离子监测对β-苯丙酮酸进行定量分析。对异构体内标物的评估。
J Chromatogr. 1978 Sep 1;146(2):213-9. doi: 10.1016/s0378-4347(00)81887-3.
6
O-trimethylsilylquinoxalinol derivatives of aromatic alpha-keto acids. Mass spectra and quantitative gas chromatography.芳香族α-酮酸的O-三甲基甲硅烷基喹喔啉醇衍生物。质谱和定量气相色谱法。
J Chromatogr. 1978 Mar 1;145(2):185-93.