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苯甲酰丙氨酸:在接受苯甲酸钠治疗的高氨血症患者中检测和鉴定与苯甲酸结合的丙氨酸

Benzoylalanine: detection and identification of an alanine conjugate with benzoic acid in hyperammonemic patients treated with sodium benzoate.

作者信息

Shinka T, Inoue Y, Kuhara T, Matsumoto M, Matsumoto I

出版信息

Clin Chim Acta. 1985 Oct 15;151(3):293-300. doi: 10.1016/0009-8981(85)90092-0.

DOI:10.1016/0009-8981(85)90092-0
PMID:4053389
Abstract

Urine of four hyperammonemic patients who were treated with large amounts of sodium benzoate were analyzed. Gas chromatographic and gas chromatograph-mass spectrometric analysis showed the presence of large amounts of benzoylalanine in addition to much hippuric acid. Benzoylalanine was identified by comparison with an authentic standard by means of its mass spectrum and the gas chromatographic retention time of its trimethylsilyl derivative. This abnormal metabolite is thought to be derived from the conjugation of benzoyl CoA with alanine. It is suggested that the excretion of benzoylalanine results from a reduction in the liver free glycine level caused by hippuric acid synthesis.

摘要

对四名接受大量苯甲酸钠治疗的高氨血症患者的尿液进行了分析。气相色谱和气相色谱 - 质谱分析表明,除了大量马尿酸外,还存在大量苯甲酰丙氨酸。通过其质谱及其三甲基硅烷基衍生物的气相色谱保留时间与 authentic standard 进行比较,鉴定出了苯甲酰丙氨酸。这种异常代谢产物被认为是由苯甲酰辅酶A与丙氨酸结合产生的。有人提出,苯甲酰丙氨酸的排泄是由于马尿酸合成导致肝脏游离甘氨酸水平降低所致。

相似文献

1
Benzoylalanine: detection and identification of an alanine conjugate with benzoic acid in hyperammonemic patients treated with sodium benzoate.苯甲酰丙氨酸:在接受苯甲酸钠治疗的高氨血症患者中检测和鉴定与苯甲酸结合的丙氨酸
Clin Chim Acta. 1985 Oct 15;151(3):293-300. doi: 10.1016/0009-8981(85)90092-0.
2
Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia.高甘氨酸血症或高氨血症患者在苯甲酸钠治疗期间马尿酸的排泄情况。
J Inherit Metab Dis. 1989;12(1):72-9. doi: 10.1007/BF01805533.
3
Methylsuccinate and mesaconate in urine of patients treated with sodium benzoate.接受苯甲酸钠治疗患者尿液中的甲基琥珀酸酯和中康酸酯。
Acta Paediatr Jpn. 1989 Dec;31(6):721-6. doi: 10.1111/j.1442-200x.1989.tb01386.x.
4
[Hypocarnitinemia in patients affected by a primary defect of ammonia metabolism treated with sodium benzoate].
Diabete Metab. 1990 May-Jun;16(3):226-33.
5
Metabolic conjugation of some carboxylic acids in the horse.马体内某些羧酸的代谢共轭作用。
Xenobiotica. 1981 Oct;11(10):655-63. doi: 10.3109/00498258109049085.
6
Effect of long-term administration of sodium benzoate to a patient with partial ornithine carbamoyl transferase deficiency.长期给一名部分鸟氨酸氨甲酰基转移酶缺乏患者服用苯甲酸钠的效果。
Clin Pediatr (Phila). 1983 Mar;22(3):206-8. doi: 10.1177/000992288302200309.
7
Effect of sodium benzoate on cerebral and hepatic energy metabolites in spf mice with congenital hyperammonemia.苯甲酸钠对先天性高氨血症SPF小鼠脑和肝能量代谢物的影响。
Biochem Pharmacol. 1993 Jan 7;45(1):137-46. doi: 10.1016/0006-2952(93)90386-b.
8
Identification of benzoylcarnitine in the urine of a patient of hyperammonemia.高氨血症患者尿液中苯甲酰肉碱的鉴定。
Tohoku J Exp Med. 1989 Oct;159(2):147-51. doi: 10.1620/tjem.159.147.
9
Treatment of hyperammonemia with sodium benzoate.
J Pediatr. 1983 Sep;103(3):498. doi: 10.1016/s0022-3476(83)80441-7.
10
Prospective management of a child with neonatal citrullinemia.新生儿瓜氨酸血症患儿的前瞻性管理。
J Pediatr. 1993 Jan;122(1):96-8. doi: 10.1016/s0022-3476(05)83497-3.

引用本文的文献

1
Benzoate treatment and the glycine index in nonketotic hyperglycinaemia.苯甲酸盐治疗与非酮症高甘氨酸血症中的甘氨酸指数
J Inherit Metab Dis. 2005;28(5):651-63. doi: 10.1007/s10545-005-0033-x.
2
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.
3
Excretion of hippuric acid during sodium benzoate therapy in patients with hyperglycinaemia or hyperammonaemia.
高甘氨酸血症或高氨血症患者在苯甲酸钠治疗期间马尿酸的排泄情况。
J Inherit Metab Dis. 1989;12(1):72-9. doi: 10.1007/BF01805533.