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伴有亚氨基二肽尿症的脯氨酰寡肽酶缺乏症：生化研究及尝试性治疗的初步结果

Prolidase deficiency with iminodipeptiduria: biochemical investigations and first results of attempted therapy.

作者信息

Charpentier C, Dagbovie K, Lemonnier A, Larregue M, Johnstone R A

出版信息

J Inherit Metab Dis. 1981;4(2):77-8. doi: 10.1007/BF02263599.

DOI:10.1007/BF02263599
PMID:6790856
Abstract

A 33-year-old female patient with chronic recurrent leg ulcerations was shown to present a massive iminodipeptiduria which seemed to be attributable to disturbance of collagen metabolism. Biochemical investigations confirmed an hereditary prolidase deficiency. A treatment was tried for the first time and showed a good biochemical result and a clinical improvement.

摘要

一名33岁患有慢性复发性腿部溃疡的女性患者被发现存在大量亚氨基二肽尿症，这似乎归因于胶原代谢紊乱。生化检查证实为遗传性脯氨酰二肽酶缺乏症。首次尝试进行治疗，结果显示生化指标良好且临床症状有所改善。

相似文献

1
Prolidase deficiency with iminodipeptiduria: biochemical investigations and first results of attempted therapy.伴有亚氨基二肽尿症的脯氨酰寡肽酶缺乏症：生化研究及尝试性治疗的初步结果
J Inherit Metab Dis. 1981;4(2):77-8. doi: 10.1007/BF02263599.
2
Iminodipeptiduria: a genetic defect in recycling collagen; a method for determining prolidase in erythrocytes.亚氨基二肽尿症：胶原蛋白循环中的一种基因缺陷；一种测定红细胞中脯氨酰二肽酶的方法。
Can Med Assoc J. 1975 Oct 18;113(8):759, 762-3.
3
Studies on a patient with iminodipeptiduria. II. Lack of prolidase activity in blood cells.
Physiol Chem Phys. 1978;10(3):279-83.
4
Prolidase deficiency.脯氨酰肽酶缺乏症
Acta Paediatr Scand. 1983 Sep;72(5):785-8. doi: 10.1111/j.1651-2227.1983.tb09815.x.
5
Further studies on a patient with iminodipeptiduria: a probable case of prolidase deficiency.
Metabolism. 1972 Dec;21(12):1113-23. doi: 10.1016/0026-0495(72)90106-0.
6
Prolidase deficiency with imidodipeptiduria. A familial case with and without clinical symptoms.伴有亚氨基二肽尿症的脯氨肽酶缺乏症。一例有临床症状和无临床症状的家族病例。
Clin Chim Acta. 1979 May 2;93(3):401-7. doi: 10.1016/0009-8981(79)90291-2.
7
Screening method for prolidase deficiency.脯氨酰肽酶缺乏症的筛查方法。
Hum Genet. 1981;56(3):349-51. doi: 10.1007/BF00274691.
8
Optimal conditions for prolidase assay by proline colorimetric determination: application to iminodipeptiduria.通过脯氨酸比色法测定脯氨肽酶的最佳条件：在亚氨基二肽尿症中的应用。
Clin Chim Acta. 1982 Oct 27;125(2):193-205. doi: 10.1016/0009-8981(82)90196-6.
9
Increased manganese content and reduced arginase activity in erythrocytes of a patient with prolidase deficiency (iminodipeptiduria).一名患有脯氨酰二肽酶缺乏症（亚氨基二肽尿症）患者红细胞中锰含量增加且精氨酸酶活性降低。
Eur J Pediatr. 1986 Apr;144(6):571-3. doi: 10.1007/BF00496038.
10
Prolidase deficiency: a patient without hydroxyproline-containing iminodipeptides in urine.脯氨酰二肽酶缺乏症：一名尿液中无含羟脯氨酸亚氨基二肽的患者。
J Inherit Metab Dis. 1988;11(2):161-5. doi: 10.1007/BF01799866.

引用本文的文献

1
Quantitative analysis of the natural history of prolidase deficiency: description of 17 families and systematic review of published cases.脯氨酸肽酶缺乏症自然病史的定量分析：17 个家系的描述及已发表病例的系统回顾
Genet Med. 2021 Sep;23(9):1604-1615. doi: 10.1038/s41436-021-01200-2. Epub 2021 May 26.
2
Prolidase directly binds and activates epidermal growth factor receptor and stimulates downstream signaling.脯氨酰内肽酶直接结合并激活表皮生长因子受体并刺激下游信号转导。
J Biol Chem. 2013 Jan 25;288(4):2365-75. doi: 10.1074/jbc.M112.429159. Epub 2012 Dec 4.
3
In-vitro responses to ascorbate and manganese in fibroblasts from a patient with prolidase deficiency and iminodipeptiduria: cell growth, prolidase activity and collagen metabolism.

本文引用的文献

1
A syndrome resembling lathyrism associated with iminodipeptiduria.一种与亚氨基二肽尿症相关的类似山黧豆中毒的综合征。
Am J Med. 1968 Jul;45(1):152-9. doi: 10.1016/0002-9343(68)90016-8.
2
A prolidase deficiency in man with iminopeptiduria.一名患有亚氨基肽尿症的男性存在脯氨酰二肽酶缺乏症。
Metabolism. 1974 Jun;23(6):505-13. doi: 10.1016/0026-0495(74)90078-x.
3
Further studies on a patient with iminodipeptiduria: a probable case of prolidase deficiency.
Metabolism. 1972 Dec;21(12):1113-23. doi: 10.1016/0026-0495(72)90106-0.
一名患有脯氨酰二肽酶缺乏症和亚氨基二肽尿症患者的成纤维细胞对抗坏血酸和锰的体外反应：细胞生长、脯氨酰二肽酶活性及胶原蛋白代谢
J Inherit Metab Dis. 1983;6(1):27-31. doi: 10.1007/BF02391189.
4
An autopsy case of prolidase deficiency.一例脯氨酰肽酶缺乏症尸检病例。
Virchows Arch A Pathol Anat Histopathol. 1985;406(1):125-31. doi: 10.1007/BF00710562.
5
Metabolic syndromes with dermatologic manifestations.伴有皮肤表现的代谢综合征
Clin Rev Allergy. 1986 Feb;4(1):101-24. doi: 10.1007/BF02991190.
6
Biochemical studies on prolidase in sera from control, patients with prolidase deficiency and their mother.
J Inherit Metab Dis. 1988;11(2):166-73. doi: 10.1007/BF01799867.
4
An ultramicrotechnique for the detection and separation of small molecular weight peptides from amino acids.一种用于从氨基酸中检测和分离小分子肽的超微技术。
Anal Biochem. 1975 Mar;64(1):260-7. doi: 10.1016/0003-2697(75)90427-3.
5
Iminopeptiduria, skin ulcerations, and edema in a boy with prolidase deficiency.
J Pediatr. 1977 Oct;91(4):578-83. doi: 10.1016/s0022-3476(77)80506-4.
6
Iminodipeptiduria: a genetic defect in recycling collagen; a method for determining prolidase in erythrocytes.亚氨基二肽尿症：胶原蛋白循环中的一种基因缺陷；一种测定红细胞中脯氨酰二肽酶的方法。
Can Med Assoc J. 1975 Oct 18;113(8):759, 762-3.
7
Prolidase deficiency: its dermatological manifestations and some additional biochemical studies.
Arch Dermatol. 1979 Jan;115(1):62-7. doi: 10.1001/archderm.115.1.62.
8
Prolidase deficiency with imidodipeptiduria. A familial case with and without clinical symptoms.伴有亚氨基二肽尿症的脯氨肽酶缺乏症。一例有临床症状和无临床症状的家族病例。
Clin Chim Acta. 1979 May 2;93(3):401-7. doi: 10.1016/0009-8981(79)90291-2.