Laminin isoforms in development and disease
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Laminin isoforms in development and disease. / Schéele, Susanne; Nyström, Alexander; Durbeej, Madeleine; Talts, Jan Fredrik; Ekblom, Marja; Ekblom, Peter.
I: Journal of Molecular Medicine, Bind 85, Nr. 8, 2007, s. 825-836.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Laminin isoforms in development and disease
AU - Schéele, Susanne
AU - Nyström, Alexander
AU - Durbeej, Madeleine
AU - Talts, Jan Fredrik
AU - Ekblom, Marja
AU - Ekblom, Peter
PY - 2007
Y1 - 2007
N2 - The members of the laminin family of heterotrimers are major constituents of all basement membranes, sheet-like extracellular structures, present in almost all organs. The laminins bind to cell surface receptors and thereby tightly connect the basement membrane to the adjacent cell layer. This provides for the specific basement membrane functions to stabilize cellular structures, to serve as effective physical barriers, and furthermore, to govern cell fate by inducing intracellular signalling cascades. Many different types of diseases involve basement membrances and laminins. Metastasizing solid tumors must pass through basement membranes to reach the vascular system, and various microbes and viruses enter the cells through direct interaction with laminins. Furthermore, whereas mutations in one specific laminin chain lead to a muscular disorder, mutations of other laminin chains cause skin blistering and kidney defects, respectively. This review summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. The current knowledge may lead to clinical treatment of lamininopathies and may include stem-cell approaches as well as gene therapy.
AB - The members of the laminin family of heterotrimers are major constituents of all basement membranes, sheet-like extracellular structures, present in almost all organs. The laminins bind to cell surface receptors and thereby tightly connect the basement membrane to the adjacent cell layer. This provides for the specific basement membrane functions to stabilize cellular structures, to serve as effective physical barriers, and furthermore, to govern cell fate by inducing intracellular signalling cascades. Many different types of diseases involve basement membrances and laminins. Metastasizing solid tumors must pass through basement membranes to reach the vascular system, and various microbes and viruses enter the cells through direct interaction with laminins. Furthermore, whereas mutations in one specific laminin chain lead to a muscular disorder, mutations of other laminin chains cause skin blistering and kidney defects, respectively. This review summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. The current knowledge may lead to clinical treatment of lamininopathies and may include stem-cell approaches as well as gene therapy.
KW - Former LIFE faculty
KW - Lamininopathies
KW - Integrin
KW - Dystroglycan
KW - Basement membranes
U2 - 10.1007/s00109-007-0182-5
DO - 10.1007/s00109-007-0182-5
M3 - Journal article
C2 - 17426950
VL - 85
SP - 825
EP - 836
JO - Journal of Molecular Medicine
JF - Journal of Molecular Medicine
SN - 0946-2716
IS - 8
ER -
ID: 8067448