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Modeling autosomal recessive cutis laxa type 1C in mice reveals distinct functions for Ltbp-4 isoforms

Recent studies have revealed an important role for LTBP-4 in elastogenesis. Its mutational inactivation in humans causes autosomal recessive cutis laxa type 1C (ARCL1C), which is a severe disorder caused by defects of the elastic fiber network. Although the human gene involved in ARCL1C has been dis...

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Furkejuvvon:
Váldodahkkit: Insa Bultmann-Mellin, Anne Conradi, Alexandra C. Maul, Katharina Dinger, Frank Wempe, Alexander P. Wohl, Thomas Imhof, F. Thomas Wunderlich, Alexander C. Bunck, Tomoyuki Nakamura, Katri Koli, Wilhelm Bloch, Alexander Ghanem, Andrea Heinz, Harald von Melchner, Gerhard Sengle, Anja Sterner-Kock
Materiálatiipa: Artigo
Giella:Inglês
Almmustuhtton: The Company of Biologists 2015-04-01
Ráidu:Disease Models & Mechanisms
Fáttát:
Latent transforming growth factor β-binding protein 4
Ltbp-4
Ltbp-4L
Ltbp-4S
Autosomal recessive cutis laxa type 1C
ARCL1C
Elastogenesis
Extracellular matrix
ECM
Fibulin-4
Fibulin-5
Liŋkkat:http://dmm.biologists.org/content/8/4/403
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