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漏斗胸肋软骨胶原蛋白的特征分析

Characterization of costal cartilage collagen in funnel chest.

作者信息

Prozorovskaya N N, Kozlov E A, Voronov A V, Verovskii V A, Delvig A A

机构信息

Institute of Biological and Medical Chemistry, Russian Academy of Medical Sciences, Moscow.

出版信息

Biomed Sci. 1991;2(6):576-80.

PMID:1841625
Abstract

Parallel measurements of pyridinoline content, the ratio of soluble:insoluble collagen, and the percentage of endogenous collagenolysis were made in samples of costal cartilage from forty-five children with funnel chest (FC) and from twenty-two control children. From an analysis of the influence of different factors, such as FC type (isolated or syndromic), a diagnosed syndrome, extent of FC depression (second or third), and age, two biochemical variants of FC-variants a and b, which were not related to the presence or absence of a known concurrent syndrome, were distinguished. These variants differed from each other in all the parameters under study. Variant a occurred about five times less frequently than b, and was characterized by a pyridinoline content of about 50% of that of b, an elevated soluble:insoluble collagen ratio, and an increased percentage of endogenous collagenolysis compared to controls. For variant b, the pyridinoline content fell within normal limits, but the soluble:insoluble collagen ratio, and the percentage of endogenous collagenolysis were below normal. The data suggest that the formation of variant a may be related to defect(s) in collagen crosslinking, whereas the formation of variant b may result from other unknown factor(s) involved in the formation and maturation of costal cartilage.

摘要

对45名漏斗胸(FC)患儿和22名对照儿童的肋软骨样本进行了吡啶啉含量、可溶性胶原蛋白与不溶性胶原蛋白的比例以及内源性胶原溶解百分比的平行测量。通过分析不同因素的影响,如FC类型(孤立型或综合征型)、诊断出的综合征、FC凹陷程度(二级或三级)和年龄,区分出了FC的两种生化变体——变体a和变体b,它们与已知并发综合征的有无无关。这些变体在所有研究参数上彼此不同。变体a出现的频率比b低约五倍,其特征是吡啶啉含量约为b的50%,可溶性胶原蛋白与不溶性胶原蛋白的比例升高,与对照组相比内源性胶原溶解百分比增加。对于变体b,吡啶啉含量在正常范围内,但可溶性胶原蛋白与不溶性胶原蛋白的比例以及内源性胶原溶解百分比低于正常水平。数据表明,变体a的形成可能与胶原交联缺陷有关,而变体b的形成可能是由于参与肋软骨形成和成熟的其他未知因素。

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