Analysis of collagen hydroxypyridinium crosslinks in samples of tissues and urine of patients with inherited connective tissue disorders.
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.
Medical and Genetic Scientific Centre, Academy of Medical Sciences of Russia, Moscow.
N V
NV
Borisova
Pokrovskaya AYa
Zakharova EYa
K D
KD
Krasnopol'skaya
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000596:Amino Acids / Q000378:metabolism* / Q000652:urine; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D003240:Connective Tissue Diseases / Q000235:genetics* / Q000378:metabolism* / Q000652:urine; D005333:Fetus / Q000378:metabolism; D006801:Humans; D007223:Infant; D008875:Middle Aged; D012016:Reference Values
D000596:Amino Acids; C015484:pyridinoline; C036020:deoxypyridinoline