Borisova N V, Pokrovskaia A Ia, Zakharova E Iu, Krasnopol'skaia K D, Poliudov S A
Biull Eksp Biol Med. 1993 May;115(5):516-8.
Ion-paired reversed-phase high performance liquid chromatography (HPLC) has been used for the analysis of the content of mature collagen crosslinks--hydroxylysylpyridinoline (HP) and lysylpyridinoline (LP) in biopsy specimens of human rib cartilage from healthy donors (n = 14) and patients with inherited diseases of the connective tissue complicated with funnel chest (n = 17). Analysis of normal tissues reveal the presence of LP (alongside with HP) in embryonal rib cartilage. LP has been found in the rib cartilage of 4 out of 6 patients with funnel chest (FC) associated with Ehlers-Danlos syndrome (EDS); with no signs of this pathology detected in individuals with isolated FC. In rib cartilage of 2 patients with recurrent isolated FC LP has been discovered alongside with the presence of type I collagen. A significant increase of LP content in rib cartilage in a child with clinical phenotype of EDS type VI has been discovered.
离子对反相高效液相色谱法(HPLC)已用于分析来自健康供体(n = 14)和患有结缔组织遗传性疾病并发漏斗胸的患者(n = 17)的人肋软骨活检标本中成熟胶原交联物——羟赖氨酰吡啶啉(HP)和赖氨酰吡啶啉(LP)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)。在6例与埃勒斯-当洛综合征(EDS)相关的漏斗胸(FC)患者中,有4例在肋软骨中发现了LP;在孤立性FC患者中未检测到这种病理迹象。在2例复发性孤立性FC患者的肋软骨中,发现LP与I型胶原同时存在。在一名具有VI型EDS临床表型的儿童的肋软骨中,发现LP含量显著增加。