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遗传性结缔组织疾病患者组织和尿液样本中胶原羟基吡啶交联的分析。

Analysis of collagen hydroxypyridinium crosslinks in samples of tissues and urine of patients with inherited connective tissue disorders.

作者信息

Borisova N V, Krasnopol'skaya K D

机构信息

Medical and Genetic Scientific Centre, Academy of Medical Sciences of Russia, Moscow.

出版信息

Connect Tissue Res. 1994;30(3):177-90. doi: 10.3109/03008209409061970.

DOI:10.3109/03008209409061970
PMID:8039385
Abstract

Ion paired reversed-phase high performance liquid chromatography in a gradient system on an Altex Ultrasphere ODS column (3 microns; 7.5 cm x 4.6 mm) protected by an Ultrasphere ODS guard cartrige (5 microns; 5.0 cm x 4.0 mm) was used for the analysis of mature collagen crosslinks-hydroxylysylpyridinoline (HP) and lysylpyridinoline (LP). The content of hydroxypyridinium crosslinks in biopsy specimens of human rib cartilage, rib bone, hip joint capsule, and urine was determined. Analysis of normal tissues revealed the presence of LP (alongside with HP) in embryonal rib cartilage and of HP+LP in the hip joint capsule. The urinary content of excreted HP in children under the age of two years exceeded the HP content in urine samples from children in the 2-12 year age group (t = 2.58, p < 0.05). The pathological material was obtained, for the most part, from patients with funnel chest (FC), both isolated and combined with the Ehlers-Danlos (EDS) and Marfan syndrome (MS). Abnormalities in tissue and urinary hydroxypyridinium crosslinks were observed in patients with clinical features of EDS. LP was found in the rib cartilage of 4 out of 6 patients with FC associated with EDS; no signs of this pathology were detected in individuals with isolated FC.

摘要

在梯度系统中,使用配备Altex Ultrasphere ODS保护柱(5微米;5.0厘米×4.0毫米)的Altex Ultrasphere ODS柱(3微米;7.5厘米×4.6毫米)进行离子对反相高效液相色谱分析,以测定成熟胶原蛋白交联物——羟赖氨酰吡啶啉(HP)和赖氨酰吡啶啉(LP)。测定了人体肋软骨、肋骨、髋关节囊活检标本及尿液中羟基吡啶交联物的含量。对正常组织的分析显示,胚胎肋软骨中存在LP(与HP一起),髋关节囊中存在HP + LP。两岁以下儿童尿液中排出的HP含量超过2 - 12岁儿童尿液样本中的HP含量(t = 2.58,p < 0.05)。病理材料大多取自患有漏斗胸(FC)的患者,包括单纯漏斗胸以及合并埃勒斯 - 当洛综合征(EDS)和马凡综合征(MS)的患者。在具有EDS临床特征的患者中观察到组织和尿液中羟基吡啶交联物异常。在6例与EDS相关的FC患者中,有4例的肋软骨中发现了LP;单纯FC患者未检测到这种病理迹象。

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Analysis of collagen hydroxypyridinium crosslinks in samples of tissues and urine of patients with inherited connective tissue disorders.遗传性结缔组织疾病患者组织和尿液样本中胶原羟基吡啶交联的分析。
Connect Tissue Res. 1994;30(3):177-90. doi: 10.3109/03008209409061970.
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[Analysis of hydroxypyridine cross-links of collagen from human rib cartilage].[人肋软骨胶原蛋白中羟基吡啶交联的分析]
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