TY - CHAP
T1 - Fibrillins
T2 - From Biogenesis of Microfibrils to Signaling Functions
AU - Hubmacher, Dirk
AU - Tiedemann, Kerstin
AU - Reinhardt, Dieter P.
N1 - Funding Information:
This work was supported by the Canadian Institutes of Health Research (MOP‐68836 to DPR) and the German Academic Exchange Service DAAD (postdoctoral fellowship to DH).
PY - 2006
Y1 - 2006
N2 - Fibrillins are large proteins that form extracellular microfibril suprastructures ubiquitously found in elastic and nonelastic tissues. Mutations in fibrillin-1 and -2 lead to a number of heritable connective tissue disorders generally termed fibrillinopathies. Clinical symptoms in fibrillinopathies manifest in the skeletal, ocular, and cardiovascular systems and highlight the importance of fibrillins in development and homeostasis of tissues and organs, including blood vessels, bone, and eye. Microfibrils appear to have dual roles in (1) conferring mechanical stability and limited elasticity to tissues, and (2) modulating the activity of growth factors of the transforming growth factor beta (TGF-β) superfamily. This chapter's focus is on the biogenesis of microfibrils, developmental expression patterns of fibrillins, signaling functions of microfibrils, and mouse models deficient in fibrillins.
AB - Fibrillins are large proteins that form extracellular microfibril suprastructures ubiquitously found in elastic and nonelastic tissues. Mutations in fibrillin-1 and -2 lead to a number of heritable connective tissue disorders generally termed fibrillinopathies. Clinical symptoms in fibrillinopathies manifest in the skeletal, ocular, and cardiovascular systems and highlight the importance of fibrillins in development and homeostasis of tissues and organs, including blood vessels, bone, and eye. Microfibrils appear to have dual roles in (1) conferring mechanical stability and limited elasticity to tissues, and (2) modulating the activity of growth factors of the transforming growth factor beta (TGF-β) superfamily. This chapter's focus is on the biogenesis of microfibrils, developmental expression patterns of fibrillins, signaling functions of microfibrils, and mouse models deficient in fibrillins.
UR - http://www.scopus.com/inward/record.url?scp=33748581008&partnerID=8YFLogxK
U2 - 10.1016/S0070-2153(06)75004-9
DO - 10.1016/S0070-2153(06)75004-9
M3 - Chapter
C2 - 16984811
AN - SCOPUS:33748581008
SN - 0121531759
SN - 9780121531751
T3 - Current Topics in Developmental Biology
SP - 93
EP - 123
BT - Current Topics in Developmental Biology
A2 - Schatten, Gerald
ER -