Molekylærbiologiske aspekter af Marfansyndromer

Tina Zimmermann Belsing; Allan Meldgaard Lund; Steen Zabell Abildstrøm; Lars Søndergaard; Lennart Friis-Hansen

Molekylærbiologiske aspekter af Marfansyndromer

Tina Zimmermann Belsing, Allan Meldgaard Lund, Steen Zabell Abildstrøm, Lars Søndergaard, Lennart Friis-Hansen

1 Citationer (Scopus)

Abstract

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.

Bidragets oversatte titel	[Molecular biological aspects of Marfan syndromes]
Originalsprog	Dansk
Tidsskrift	Ugeskrift for Laeger
Vol/bind	173
Udgave nummer	5
Sider (fra-til)	333-7
Antal sider	5
ISSN	0041-5782
Status	Udgivet - jan. 2011

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http://www.ugeskriftet.dk.ep.fjernadgang.kb.dk/portal/page/portal/LAEGERDK/UGESKRIFT_FOR_LAEGER/Artikelvisning?pUrl=/UGESKRIFT_FOR_LAEGER/TIDLIGERE_NUMRE/2011/UFL_2011_5/UFL_2011_173_5_333

Citationsformater

Belsing, TZ, Lund, AM, Abildstrøm, SZ, Søndergaard, L & Friis-Hansen, L 2011, 'Molekylærbiologiske aspekter af Marfansyndromer', Ugeskrift for Laeger, bind 173, nr. 5, s. 333-7. <http://www.ugeskriftet.dk.ep.fjernadgang.kb.dk/portal/page/portal/LAEGERDK/UGESKRIFT_FOR_LAEGER/Artikelvisning?pUrl=/UGESKRIFT_FOR_LAEGER/TIDLIGERE_NUMRE/2011/UFL_2011_5/UFL_2011_173_5_333>

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title = "Molekyl{\ae}rbiologiske aspekter af Marfansyndromer",

abstract = "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.",

author = "Belsing, {Tina Zimmermann} and Lund, {Allan Meldgaard} and Abildstr{\o}m, {Steen Zabell} and Lars S{\o}ndergaard and Lennart Friis-Hansen",

year = "2011",

month = jan,

language = "Dansk",

volume = "173",

pages = "333--7",

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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 - 2011/1

Y1 - 2011/1

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

SN - 0041-5782

VL - 173

SP - 333

EP - 337

JO - Ugeskrift for Laeger

JF - Ugeskrift for Laeger

IS - 5

ER -

Molekylærbiologiske aspekter af Marfansyndromer

Abstract

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