SYNTHESIS OF AN ALTERED TYPE-III PROCOLLAGEN IN A PATIENT WITH TYPE-IV EHLERS-DANLOS SYNDROME - A STRUCTURAL-CHANGE IN THE ALPHA-1(III) CHAIN WHICH MAKES THE PROTEIN MORE SUSCEPTIBLE TO PROTEINASES

被引：0

作者：

STOLLE, CA

PYERITZ, RE

MYERS, JC

PROCKOP, DJ

机构：

[1] UNIV MED & DENT NEW JERSEY, RUTGERS MED SCH, DEPT BIOCHEM, PISCATAWAY, NJ 08854 USA

[2] JOHNS HOPKINS UNIV, SCH MED, DEPT MED, BALTIMORE, MD 21205 USA

[3] JOHNS HOPKINS UNIV, SCH MED, DEPT PEDIAT, BALTIMORE, MD 21205 USA

来源：

JOURNAL OF BIOLOGICAL CHEMISTRY | 1985年 / 260卷 / 03期

关键词：

D O I：

暂无

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：

引用

页码：1937 / 1944

页数：8

共 50 条

[21] DETECTION OF TYPE-III COLLAGEN IN SKIN FIBROBLASTS FROM PATIENTS WITH EHLERS-DANLOS SYNDROME TYPE-IV BY IMMUNOFLUORESCENCE
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[22] A GLY(415) TO SER SUBSTITUTION RESULTS IN IMPAIRED SECRETION AND DECREASED THERMAL-STABILITY OF TYPE-III PROCOLLAGEN IN A PATIENT WITH EHLERS-DANLOS SYNDROME TYPE-IV
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[23] EHLERS-DANLOS SYNDROME TYPE-IV - A SUBSET OF PATIENTS DISTINGUISHED BY LOW SERUM LEVELS OF THE AMINO-TERMINAL PROPEPTIDE OF TYPE-III PROCOLLAGEN
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[25] BIOCHEMICAL AND IMMUNOLOGICAL STUDIES OF FIBROBLASTS DERIVED FROM A PATIENT WITH EHLERS-DANLOS SYNDROME TYPE-IV DEMONSTRATE REDUCED TYPE-III COLLAGEN-SYNTHESIS
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[26] A SINGLE BASE MUTATION THAT SUBSTITUTES SERINE FOR GLYCINE-790 OF THE ALPHA-1(III) CHAIN OF TYPE-III PROCOLLAGEN EXPOSES AN ARGININE AND CAUSES EHLERS-DANLOS SYNDROME .4.
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[27] A BASE SUBSTITUTION AT A SPLICE SITE IN THE COL3A1 GENE CAUSES EXON SKIPPING AND GENERATES ABNORMAL TYPE-III PROCOLLAGEN IN A PATIENT WITH EHLERS-DANLOS SYNDROME TYPE-IV
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