Biallelic mutations in LAMA5 disrupts a skeletal noncanonical focal adhesion pathway and produces a distinct bent bone dysplasia

Biallelic mutations in LAMA5 disrupts a skeletal noncanonical focal adhesion pathway and produces a distinct bent bone dysplasia

Background: Beyond its structural role in the skeleton, the extracellular matrix (ECM), particularly basement membrane proteins, facilitates communication with intracellular signaling pathways and cell to cell interactions to control differentiation, proliferation, migration and survival. Alteration...

Full description

Bibliographic Details
Main Authors: Maya Barad, Fabiana Csukasi, Michaela Bosakova, Jorge H. Martin, Wenjuan Zhang, S. Paige Taylor, Ralph S. Lachman, Jennifer Zieba, Michael Bamshad, Deborah Nickerson, Jessica X. Chong, Daniel H. Cohn, Pavel Krejci, Deborah Krakow, Ivan Duran
Format: Article
Language:English
Published: Elsevier 2020-12-01
Series:EBioMedicine
Subjects:
Laminin α5
LAMA5
Skeletal dysplasia
Bent bone
β1 integrin
Online Access:http://www.sciencedirect.com/science/article/pii/S2352396420304515

Internet

http://www.sciencedirect.com/science/article/pii/S2352396420304515

Similar Items