Stone P J, Korn J H, North H, Lally E V, Miller L C, Tucker L B, Strongwater S, Snider G L, Franzblau C
Biochemistry Department, Boston University School of Medicine, MA 02118, USA.
Arthritis Rheum. 1995 Apr;38(4):517-24. doi: 10.1002/art.1780380409.
To measure the urinary excretion of specific cross-link amino acid markers for mature elastin (desmosine [DES] and isodesmosine [IDES]) and fibrillar collagen (hydroxylysylpyridinoline [HP] and lysylpyridinoline [LP]) in systemic sclerosis (SSc) patients and healthy controls.
Urine specimens from 20 patients with SSc and 22 controls were assessed for DES, IDES, HP, and LP using high performance liquid chromatography and ultraviolet absorption spectroscopy, in combination with an isotope dilution technique in which the urine specimen was spiked with isotopically labeled cross-link amino acids.
Mean +/- SD levels of urinary DES and IDES were elevated in SSc patients by 2-3-fold, and urinary HP and LP by 3-4-fold, compared with controls (DES 21.0 +/- 9.4 versus 7.5 +/- 1.4 micrograms/gm creatinine; HP 109.0 +/- 72.9 versus 24.9 +/- 5.7 nmoles/mmole creatinine). Nineteen of the 20 SSc patients had urinary DES and HP values that were > 3 SD above the control mean. A significant elevation in the HP:LP ratio in SSc patients as compared with controls (mean +/- SD 6.9 +/- 1.5 versus 5.5 +/- 1.3) indicated a soft tissue origin for much of the increased HP.
Patients with SSc have higher levels of urinary cross-link amino acids specific for the degradation of mature collagen and elastin. These markers distinguish most SSc patients from healthy controls.
测量系统性硬化症（SSc）患者及健康对照者尿液中成熟弹性蛋白特异性交联氨基酸标志物（锁链素[DES]和异锁链素[IDES]）以及纤维状胶原蛋白（羟赖氨酰吡啶啉[HP]和赖氨酰吡啶啉[LP]）的排泄量。
采用高效液相色谱法和紫外吸收光谱法，并结合同位素稀释技术（向尿液标本中加入同位素标记的交联氨基酸），对20例SSc患者和22例对照者的尿液标本进行DES、IDES、HP和LP检测。
与对照组相比，SSc患者尿液中DES和IDES的平均±标准差水平升高了2至3倍，尿液中HP和LP升高了3至4倍（DES：21.0±9.4对7.5±1.4微克/克肌酐；HP：109.0±72.9对24.9±5.7纳摩尔/毫摩尔肌酐）。20例SSc患者中有19例的尿液DES和HP值高于对照平均值3个标准差。与对照组相比，SSc患者HP:LP比值显著升高（平均±标准差6.9±1.5对5.5±1.3），表明大部分升高的HP来源于软组织。
SSc患者尿液中成熟胶原蛋白和弹性蛋白降解特异性交联氨基酸水平较高。这些标志物可将大多数SSc患者与健康对照者区分开来。
