Molekylærbiologiske aspekter af Marfansyndromer
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Molekylærbiologiske aspekter af Marfansyndromer. / Belsing, Tina Zimmermann; Lund, Allan Meldgaard; Abildstrøm, Steen Zabell; Søndergaard, Lars; Friis-Hansen, Lennart.
I: Ugeskrift for Laeger, Bind 173, Nr. 5, 2011, s. 333-7.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Molekylærbiologiske aspekter af Marfansyndromer
AU - Belsing, Tina Zimmermann
AU - Lund, Allan Meldgaard
AU - Abildstrøm, Steen Zabell
AU - Søndergaard, Lars
AU - Friis-Hansen, Lennart
PY - 2011
Y1 - 2011
N2 - Marfan syndrome (MFS) is a hereditary connective tissue disorder. Studies of MFS have established the critical contribution of fibrillin-1 deficiency to disease progression through altered cell-matrix interactions and dysregulated TGF-ß signalling. It is now known that the disease is caused by altered regulation of TGF-ß. As a result, the definition of MFS- and MFS-related diseases as the prototypical structural disorder of the connective tissue has changed to that of a developmental abnormality with broad and complex effects on the morphogenesis and tissue remodelling.
AB - Marfan syndrome (MFS) is a hereditary connective tissue disorder. Studies of MFS have established the critical contribution of fibrillin-1 deficiency to disease progression through altered cell-matrix interactions and dysregulated TGF-ß signalling. It is now known that the disease is caused by altered regulation of TGF-ß. As a result, the definition of MFS- and MFS-related diseases as the prototypical structural disorder of the connective tissue has changed to that of a developmental abnormality with broad and complex effects on the morphogenesis and tissue remodelling.
M3 - Tidsskriftartikel
VL - 173
SP - 333
EP - 337
JO - Ugeskrift for Laeger
JF - Ugeskrift for Laeger
SN - 0041-5782
IS - 5
ER -
ID: 40176136