Stiff and Tight Skin: A Rear Window into Fibrosis Without Inflammation

被引:7
作者
Avvedimento, Enrico V. [1 ]
Gabrielli, Armando [2 ]
机构
[1] Univ Naples Federico II, Sch Med, Dept Biol & Patol Mol & Cellulare, Naples, Italy
[2] Univ Politecn Marche, Sez Clin Med, Dept Sci Med & Chirurg, Ancona, Italy
关键词
GROWTH-FACTOR; MARFAN-SYNDROME; FIBRILLIN; MATRIX;
D O I
10.1126/scitranslmed.3000949
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
In this issue of Science Translational Medicine, a report by Loeys et al. on mutations in the fibrillin-1 gene in patients with skin fibrosis (stiff skin) adds a new piece of information on a connective tissue disorder that resembles systemic sclerosis, an autoimmune disease characterized by skin fibrosis and visceral organ involvement. Here, we discuss the implications of these findings, as well as new opportunities for targeted therapy for fibrosis.
引用
收藏
页数:3
相关论文
共 21 条
[1]   The integrin - Growth factor receptor duet [J].
Alam, Naved ;
Goel, Hira Lal ;
Zarif, Matthew J. ;
Butterfield, Julie E. ;
Perkins, Hillary M. ;
Sansoucy, Brian G. ;
Sawyer, Thomas K. ;
Languino, Lucia R. .
JOURNAL OF CELLULAR PHYSIOLOGY, 2007, 213 (03) :649-653
[2]   Genetic diseases of connective tissues: cellular and extracellular effects of ECM mutations [J].
Bateman, John F. ;
Boot-Handford, Raymond P. ;
Lamande, Shireen R. .
NATURE REVIEWS GENETICS, 2009, 10 (03) :173-183
[3]   Increased expression of Wnt2 and SFRP4 in Tsk mouse skin: Role of Wnt signaling in altered dermal fibrillin deposition and systemic sclerosis [J].
Bayle, Julie ;
Fitch, Jennifer ;
Jacobsen, Kimberly ;
Kumar, Rajiv ;
Lafyatis, Robert ;
Lemaire, Raphael .
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 2008, 128 (04) :871-881
[4]   p38 MAPK Is an Early Determinant of Promiscuous Smad2/3 Signaling in the Aortas of Fibrillin-1 (Fbn1)-null Mice [J].
Carta, Luca ;
Smaldone, Silvia ;
Zilberberg, Lior ;
Loch, David ;
Dietz, Harry C. ;
Rifkin, Daniel B. ;
Ramirez, Francesco .
JOURNAL OF BIOLOGICAL CHEMISTRY, 2009, 284 (09) :5630-5636
[5]   Molecular Framework for Response to Imatinib Mesylate in Systemic Sclerosis [J].
Chung, Lorinda ;
Fiorentino, David F. ;
BenBarak, Maya J. ;
Adler, Adam S. ;
Mariano, Melissa M. ;
Paniagua, Ricardo T. ;
Milano, Ausra ;
Connolly, M. Kari ;
Ratiner, Boris D. ;
Wiskocil, Robert L. ;
Whitfield, Michael L. ;
Chang, Howard Y. ;
Robinson, William H. .
ARTHRITIS AND RHEUMATISM, 2009, 60 (02) :584-591
[6]   Tyrosine kinase inhibitors for the treatment of fibrotic diseases such as systemic sclerosis: towards molecular targeted therapies [J].
Distler, J. H. W. ;
Distler, O. .
ANNALS OF THE RHEUMATIC DISEASES, 2010, 69 :48-51
[7]  
GABRIELLI A, 1989, NEW ENGL J MED, V360, P2003
[8]  
Hinz Boris, 2009, Curr Rheumatol Rep, V11, P120
[9]   The Extracellular Matrix: Not Just Pretty Fibrils [J].
Hynes, Richard O. .
SCIENCE, 2009, 326 (5957) :1216-1219
[10]   Latent transforming growth factor β-binding protein 1 interacts with fibrillin and is a microfibril-associated protein [J].
Isogai, Z ;
Ono, RN ;
Ushiro, S ;
Keene, DR ;
Chen, Y ;
Mazzieri, R ;
Charbonneau, NL ;
Reinhardt, DP ;
Rifkin, DB ;
Sakai, LY .
JOURNAL OF BIOLOGICAL CHEMISTRY, 2003, 278 (04) :2750-2757