Stiff and tight skin: a rear window into fibrosis without inflammation.
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.
Avvedimento
Enrico V
EV
Department of Biologia e Patologia Molecolare e Cellulare, Universita Federico II Medical School, Naples, Italy. avvedim@unina.it
Gabrielli
Armando
A
D000818:Animals; D005109:Extracellular Matrix / Q000378:metabolism / Q000473:pathology; D000071838:Fibrillin-1; D000071837:Fibrillins; D005355:Fibrosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D006801:Humans; D007249:Inflammation / Q000235:genetics / Q000473:pathology; D008382:Marfan Syndrome / Q000235:genetics / Q000473:pathology; D051379:Mice; D008840:Microfilament Proteins / Q000235:genetics; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D051785:Smad Proteins / Q000378:metabolism; D054392:Adipokines
D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; D051785:Smad Proteins; C000605722:FBN1 protein, human; D054392:Adipokines; C000605724:Fbn1 protein, mouse