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以5-氧脯氨酸的尿排泄量(焦谷氨酸尿症)作为正常人甘氨酸不足的指标。

Urinary excretion of 5-oxoproline (pyroglutamic aciduria) as an index of glycine insufficiency in normal man.

作者信息

Jackson A A, Badaloo A V, Forrester T, Hibbert J M, Persaud C

机构信息

Tropical Metabolism Research Unit, University of the West Indies, Mona, Kingston, Jamaica.

出版信息

Br J Nutr. 1987 Sep;58(2):207-14. doi: 10.1079/bjn19870088.

DOI:10.1079/bjn19870088
PMID:3676243
Abstract
  1. The evidence is accumulating to suggest that glycine, the simplest amino acid, is conditionally essential in man. Benzoic acid, by conjugation with glycine to form hippuric acid, is known to deplete the free glycine pool of the body. Glycine is one substrate for the enzyme glutathione synthase (EC 6.3.2.3) and in the inborn error of metabolism in which glutathione synthase function is defective, increased quantities of 5-oxoproline are excreted in the urine. 2. An oral dose of 4-10 g sodium benzoate was given to six normal adults to deplete the metabolic pool of glycine, and the urinary excretion of 5-oxoproline was followed for 6 h. In five of the six, a significant increase in the urinary 5-oxoproline was seen within 3 h. 3. These findings show that 5-oxoprolinuria can result from limited glycine availability, and may provide a useful test for assessing glycine sufficiency in a range of physiological and pathological states.
摘要
  1. 越来越多的证据表明,最简单的氨基酸甘氨酸在人体中是条件必需的。苯甲酸通过与甘氨酸结合形成马尿酸,已知会耗尽人体的游离甘氨酸池。甘氨酸是谷胱甘肽合酶(EC 6.3.2.3)的一种底物,在谷胱甘肽合酶功能有缺陷的先天性代谢异常中,尿中5-氧代脯氨酸的排泄量会增加。2. 给6名正常成年人口服4-10克苯甲酸钠以耗尽甘氨酸的代谢池,并对尿中5-氧代脯氨酸的排泄情况进行了6小时的跟踪。在6人中的5人身上,在3小时内尿中5-氧代脯氨酸出现了显著增加。3. 这些发现表明,5-氧代脯氨酸尿症可能是由于甘氨酸供应有限导致的,并且可能为评估一系列生理和病理状态下的甘氨酸充足情况提供一种有用的检测方法。

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1
Urinary excretion of 5-oxoproline (pyroglutamic aciduria) as an index of glycine insufficiency in normal man.以5-氧脯氨酸的尿排泄量(焦谷氨酸尿症)作为正常人甘氨酸不足的指标。
Br J Nutr. 1987 Sep;58(2):207-14. doi: 10.1079/bjn19870088.
2
Urinary excretion of 5-L-oxoproline (pyroglutamic acid) is increased during recovery from severe childhood malnutrition and responds to supplemental glycine.在严重儿童营养不良恢复期间,5-L-氧代脯氨酸(焦谷氨酸)的尿排泄量增加,且对补充甘氨酸有反应。
J Nutr. 1996 Nov;126(11):2823-30. doi: 10.1093/jn/126.11.2823.
3
On the mechanism of 5-oxoproline overproduction in 5-oxoprolinuria.关于5-氧脯氨酸尿症中5-氧脯氨酸过度产生的机制。
Clin Chim Acta. 1976 Mar 15;67(3):245-53. doi: 10.1016/0009-8981(76)90332-6.
4
The excretion of 5-oxoproline in urine, as an index of glycine status, during normal pregnancy.
Br J Obstet Gynaecol. 1989 Apr;96(4):440-4. doi: 10.1111/j.1471-0528.1989.tb02420.x.
5
Glutathione synthetase deficiency, an inborn error of metabolism involving the gamma-glutamyl cycle in patients with 5-oxoprolinuria (pyroglutamic aciduria).谷胱甘肽合成酶缺乏症,一种涉及5-氧脯氨酸尿症(焦谷氨酸尿症)患者γ-谷氨酰循环的先天性代谢缺陷。
Proc Natl Acad Sci U S A. 1974 Jun;71(6):2505-9. doi: 10.1073/pnas.71.6.2505.
6
Urinary excretion of 5-L-oxoproline (pyroglutamic acid) during early life in term and preterm infants.足月儿和早产儿生命早期5-L-氧代脯氨酸(焦谷氨酸)的尿排泄情况。
Arch Dis Child Fetal Neonatal Ed. 1997 May;76(3):F152-7. doi: 10.1136/fn.76.3.f152.
7
Oxoproline kinetics and oxoproline urinary excretion during glycine- or sulfur amino acid-free diets in humans.人体在无甘氨酸或无硫氨基酸饮食期间的氧脯氨酸动力学和氧脯氨酸尿排泄情况。
Am J Physiol Endocrinol Metab. 2000 May;278(5):E868-76. doi: 10.1152/ajpendo.2000.278.5.E868.
8
Neonatal 5-oxoprolinuria: difficult-to-diagnose?
J Inherit Metab Dis. 1983;6(1):44-8. doi: 10.1007/BF02391193.
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Infants in Trinidad excrete more 5-L-oxoproline (L-pyroglutamic acid) in urine than infants in England: an environmental not ethnic difference.特立尼达的婴儿尿液中排泄的5-L-氧代脯氨酸(L-焦谷氨酸)比英国婴儿多:这是环境差异而非种族差异。
Br J Nutr. 1998 Jul;80(1):51-5. doi: 10.1017/s0007114598001767.
10
5-oxoprolinuria due to hereditary 5-oxoprolinase deficiency in two brothers--a new inborn error of the gamma-glutamyl cycle.两兄弟因遗传性5-氧脯氨酸酶缺乏导致5-氧脯氨酸尿症——γ-谷氨酰循环中的一种新的先天性代谢缺陷。
Acta Paediatr Scand. 1981;70(3):301-8. doi: 10.1111/j.1651-2227.1981.tb16556.x.

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