CROSS-LINKING OF COLLAGEN IN THE X-LINKED EHLERS-DANLOS TYPE-V

被引:43
作者
SIEGEL, RC
BLACK, CM
BAILEY, AJ
机构
[1] HAMMERSMITH HOSP,ROYAL POSTGRAD MED SCH,LONDON W12 0HS,ENGLAND
[2] ARC,INST MEAT RES,BRISTOL BS18 7DY,AVON,ENGLAND
关键词
D O I
10.1016/0006-291X(79)91727-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The activity and antigenicity of the collagen crosslinking enzyme, lysyl oxidase, and the proportions of reducible crosslink in skin biopsies from Ehlers-Danlos Type V subjects were equivalent to those of control skin. These results reveal that both the potential for crosslinking, and the ability to form reducible crosslinks is present in Ehlers-Danlos syndrome Type V subjects, clearly demonstrating that the defect in this disorder is not due to a defective crosslinking mechanism. © 1979.
引用
收藏
页码:281 / 287
页数:7
相关论文
共 18 条
[1]  
BEIGHTON P, 1970, EHLERS DANLOS SYNDRO, P29
[2]  
BLACK CM, 1978, ARTHRITIS RHEUM, V21, P545
[4]  
DIFERRANTE N, 1975, CONNECT TISSUE RES, V3, P49
[5]  
EUNDUCK P, 1975, IMMUNOLOGY, V28, P781
[6]   PHYSICAL PROPERTIES OF SKIN IN EHLERS-DANLOS SYNDROME [J].
GRAHAME, R ;
BEIGHTON, P .
ANNALS OF THE RHEUMATIC DISEASES, 1969, 28 (03) :246-&
[7]   LYSYL-PROTOCOLLAGEN HYDROXYLASE DEFICIENCY IN FIBROBLASTS FROM SIBLINGS GS WITH HYDROXYLYSINE-DEFICIENT COLLAGEN [J].
KRANE, SM ;
PINNELL, SR ;
ERBE, RW .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1972, 69 (10) :2899-&
[8]  
LAPIERE CM, 1976, BIOCH COLLAGEN, P377
[9]   PRODUCTION OF LYSYL OXIDASE BY HUMAN FIBROBLASTS IN CULTURE [J].
LAYMAN, DL ;
MARTIN, GR ;
NARAYANAN, AS .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1972, 149 (01) :97-+
[10]   DEFECT IN CONVERSION OF PROCOLLAGEN TO COLLAGEN IN A FORM OF EHLERS-DANLOS SYNDROME [J].
LICHTENSTEIN, JR ;
MARTIN, GR ;
KOHN, LD ;
BYERS, PH ;
MCKUSICK, VA .
SCIENCE, 1973, 182 (4109) :298-300