HYDROXYLATION OF COLLAGEN TYPE-I - EVIDENCE THAT BOTH LYSYL AND PROLYL RESIDUES ARE OVERHYDROXYLATED IN OSTEOGENESIS IMPERFECTA

被引：10
作者：
LEHMANN, HW
RIMEK, D
BODO, M
BRENNER, RE
VETTER, U
WORSDORFER, O
KARBOWSKI, A
MULLER, PK
机构：
[1] UNIV LUBECK,INST MED MOLEK BIOL,W-2400 LUBECK,GERMANY
[2] UNIV ULM,ZENTRUM KINDERHEILKUNDE,W-7900 ULM,GERMANY
[3] FREE UNIV BERLIN,KLINIKUM CHARITE,BERLIN,GERMANY
[4] UNFALLCHIRURG ORTHOPAD KLIN,FULDA,GERMANY
[5] UNIV MAINZ,ORTHOPAD KLIN,W-6500 MAINZ,GERMANY
来源：
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION | 1995年 / 25卷 / 05期
关键词：
HYDROXYLYSINE; HYDROXYPROLINE; OSTEOGENESIS IMPERFECTA;
D O I：
10.1111/j.1365-2362.1995.tb01706.x
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
The composition of the collagens secreted into the media of fibroblast cultures of 39 patients with osteogenesis imperfecta (OI) was the same in controls and OI cultures. An abnormal migration pattern of collagens upon SDS-PAGE was evident in one third of the cultures investigated. Lysyl and prolyl hydroxylation of HPLC-purified alpha 1(I) chains was elevated in about 60% of cultures. The degree of hydroxylation was highest in the lethal forms. The extent of lysyl and prolyl hydroxylation showed a strong correlation (r = 0.74, P < 0.001). While high levels of hydroxylation are frequently observed in OI patients, a direct correlation between lysyl of prolyl hydroxylation and fracture rate or growth retardation could not be established.
引用
收藏
页码：306 / 310
页数：5
相关论文
共 17 条
[1]   FOLDING MECHANISM OF THE TRIPLE HELIX IN TYPE-III COLAGEN AND TYPE-III PN-COLLAGEN - ROLE OF DISULFIDE BRIDGES AND PEPTIDE-BOND ISOMERIZATION [J].
BACHINGER, HP ;
BRUCKNER, P ;
TIMPL, R ;
PROCKOP, DJ ;
ENGEL, J .
EUROPEAN JOURNAL OF BIOCHEMISTRY, 1980, 106 (02) :619-632
[2]   ABNORMAL TYPE-I COLLAGEN-METABOLISM BY CULTURED FIBROBLASTS IN LETHAL PERINATAL OSTEOGENESIS IMPERFECTA [J].
BATEMAN, JF ;
MASCARA, T ;
CHAN, D ;
COLE, WG .
BIOCHEMICAL JOURNAL, 1984, 217 (01) :103-115
[3]   OSTEOGENESIS IMPERFECTA - INSUFFICIENT COLLAGEN-SYNTHESIS IN EARLY-CHILDHOOD AS EVIDENCED BY ANALYSIS OF COMPACT-BONE AND FIBROBLAST-CULTURES [J].
BRENNER, RE ;
VETTER, U ;
NERLICH, A ;
WORSDORFER, O ;
TELLER, WM ;
MULLER, PK .
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, 1989, 19 (02) :159-166
[4]   3 CONFORMATIONALLY DISTINCT DOMAINS IN AMINO-TERMINAL SEGMENT OF TYPE-III PROCOLLAGEN AND ITS RAPID TRIPLE HELIX REVERSIBLE COIL TRANSITION [J].
BRUCKNER, P ;
BACHINGER, HP ;
TIMPL, R ;
ENGEL, J .
EUROPEAN JOURNAL OF BIOCHEMISTRY, 1978, 90 (03) :595-603
[5]  
Byers Peter H., 1993, P317
[6]   THE ZIPPER-LIKE FOLDING OF COLLAGEN TRIPLE HELICES AND THE EFFECTS OF MUTATIONS THAT DISRUPT THE ZIPPER [J].
ENGEL, J ;
PROCKOP, DJ .
ANNUAL REVIEW OF BIOPHYSICS AND BIOPHYSICAL CHEMISTRY, 1991, 20 :137-152
[7]   A TYPE-I COLLAGEN WITH SUBSTITUTION OF A CYSTEINE FOR GLYCINE-748 IN THE ALPHA-1(I) CHAIN COPOLYMERIZES WITH NORMAL TYPE-I COLLAGEN AND CAN GENERATE FRACTALLIKE STRUCTURES [J].
KADLER, KE ;
TORREBLANCO, A ;
ADACHI, E ;
VOGEL, BE ;
HOJIMA, Y ;
PROCKOP, DJ .
BIOCHEMISTRY, 1991, 30 (20) :5081-5088
[8]  
KIVIRIKKO KI, 1984, EXTRACELLULAR MATRIX, P83
[9]   MUTATIONS IN COLLAGEN GENES - CAUSES OF RARE AND SOME COMMON DISEASES IN HUMANS [J].
KUIVANIEMI, H ;
TROMP, G ;
PROCKOP, DJ .
FASEB JOURNAL, 1991, 5 (07) :2052-2060
[10]   LYSYL HYDROXYLATION IN COLLAGENS FROM HYPERPLASTIC CALLUS AND EMBRYONIC BONES [J].
LEHMANN, HW ;
BODO, M ;
FROHN, C ;
NERLICH, A ;
RIMEK, D ;
NOTBOHM, H ;
MULLER, PK .
BIOCHEMICAL JOURNAL, 1992, 282 :313-318
12