Molecular and cellular basis of scleroderma

被引:40
作者
Eckes, Beate [1 ]
Moinzadeh, Pia [1 ]
Sengle, Gerhard [2 ,3 ]
Hunzelmann, Nico [1 ]
Krieg, Thomas [1 ,3 ,4 ]
机构
[1] Univ Cologne, Dept Dermatol, D-50937 Cologne, Germany
[2] Univ Cologne, Ctr Biochem, D-50937 Cologne, Germany
[3] Univ Cologne, CMMC, D-50937 Cologne, Germany
[4] Univ Cologne, Cologne Excellence Cluster Cellular Stress Respon, D-50937 Cologne, Germany
来源
JOURNAL OF MOLECULAR MEDICINE-JMM | 2014年 / 92卷 / 09期
关键词
Systemic sclerosis; Scleroderma; Extracellular microenvironment; Pathophysiology; Signaling; CUTANEOUS SYSTEMIC-SCLEROSIS; GENOME-WIDE ASSOCIATION; GROWTH-FACTOR RECEPTOR; CONNECTIVE-TISSUE; EXTRACELLULAR-MATRIX; GENE-EXPRESSION; STIMULATORY AUTOANTIBODIES; DISEASE SCLERODERMA; STRUCTURAL-ANALYSIS; COLLAGEN-SYNTHESIS;
D O I
10.1007/s00109-014-1190-x
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Systemic sclerosis (scleroderma) is a chronic inflammatory disease that leads to fibrosis of the skin and involved internal organs. No efficient therapy is currently available. This review summarizes recent progress made in basic as well as clinical science and concludes with a concept that therapy targeting fibrosis in scleroderma needs to take into account the global microenvironment in the skin with its diverse cellular players interacting with a complex extracellular matrix environment and matrix-associated growth factors.
引用
收藏
页码:913 / 924
页数:12
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