Function of Ltbp-4L and fibulin-4 in survival and elastogenesis in mice

Function of Ltbp-4L and fibulin-4 in survival and elastogenesis in mice

LTBP-4L and LTBP-4S are two isoforms of the extracellular matrix protein latent-transforming growth factor beta-binding protein 4 (LTBP-4). The mutational inactivation of both isoforms causes autosomal recessive cutis laxa type 1C (ARCL1C) in humans and an ARCL1C-like phenotype in Ltbp4−/− mice, bot...

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Bibliographic Details
Main Authors: Insa Bultmann-Mellin, Jeroen Essers, Paula M. van Heijingen, Harald von Melchner, Gerhard Sengle, Anja Sterner-Kock
Format: Article
Language:English
Published: The Company of Biologists 2016-11-01
Series:Disease Models & Mechanisms
Subjects:
Latent-transforming growth factor beta-binding protein 4 (Ltbp-4)
Fibulin-4
Elastic fibers
Defective alveolar septation
Aortic tortuosity
Online Access:http://dmm.biologists.org/content/9/11/1367

Internet

http://dmm.biologists.org/content/9/11/1367

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