TY - JOUR
T1 - Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development
AU - Quondamatteo, Fabio
AU - Reinhardt, Dieter P.
AU - Charbonneau, Noe L.
AU - Pophal, Gabriele
AU - Sakai, Lynn Y.
AU - Herken, Rainer
N1 - Funding Information:
The authors thank Robert N. Ono and Glen Corson, Shriners Hospital for Children, Portland, OR, for carrying out the experiments which excluded cross-reactivity of the mAb 48 vs. fibrillin-3. The authors are also grateful to Elke Heyder for technical assistance and to Cyrilla Maelicke for editing the manuscript. This work was supported by grants from the Deutsche Forschungsgemeinschaft (Re 1021/3-1 and 1021/4-1 to D.P.R.).
PY - 2002/12
Y1 - 2002/12
N2 - The extracellular glycoproteins fibrillin-1 and fibrillin-2 are major components of connective tissue microfibrils. Mutations in the fibrillin-1 and fibrillin-2 genes are responsible for the phenotypical manifestations of Marfan syndrome and congenital contractural arachnodactyly respectively, which emphasizes their essential roles in developmental processes of various tissues. Consistent with this last notion, organ culture experiments have indirectly suggested morphogenic roles for fibrillins in lung and kidney development. In order to contribute to the understanding of the roles of fibrillins in developmental and morphogenetic events, we have investigated the distribution of fibrillin-1 and fibrillin-2 in human embryonic and early fetal tissues between the 5th and the 12th gestational week, i.e. at the beginning of organogenesis. Fibrillin-1 and fibrillin-2 were localized immunohistochemically using specific monoclonal antibodies, mAb 69 and mAb 48, respectively. Both fibrillins are widely distributed in various human anlagen, from early developmental stages. In most embryonic and early fetal human organs such as skin, lung, heart, aorta, central nervous system anlage, nerves, and ganglia, fibrillin-1 and fibrillin-2 follow the same temporo-spatial pattern of distribution. However, in other organs such as kidney, liver, rib anlagen, notochord fibrillin-1 and fibrillin-2 are distributed differentially. The present paper is focused on this aspect. These results suggest different roles for fibrillin-1 and -2 in the development of these structures.
AB - The extracellular glycoproteins fibrillin-1 and fibrillin-2 are major components of connective tissue microfibrils. Mutations in the fibrillin-1 and fibrillin-2 genes are responsible for the phenotypical manifestations of Marfan syndrome and congenital contractural arachnodactyly respectively, which emphasizes their essential roles in developmental processes of various tissues. Consistent with this last notion, organ culture experiments have indirectly suggested morphogenic roles for fibrillins in lung and kidney development. In order to contribute to the understanding of the roles of fibrillins in developmental and morphogenetic events, we have investigated the distribution of fibrillin-1 and fibrillin-2 in human embryonic and early fetal tissues between the 5th and the 12th gestational week, i.e. at the beginning of organogenesis. Fibrillin-1 and fibrillin-2 were localized immunohistochemically using specific monoclonal antibodies, mAb 69 and mAb 48, respectively. Both fibrillins are widely distributed in various human anlagen, from early developmental stages. In most embryonic and early fetal human organs such as skin, lung, heart, aorta, central nervous system anlage, nerves, and ganglia, fibrillin-1 and fibrillin-2 follow the same temporo-spatial pattern of distribution. However, in other organs such as kidney, liver, rib anlagen, notochord fibrillin-1 and fibrillin-2 are distributed differentially. The present paper is focused on this aspect. These results suggest different roles for fibrillin-1 and -2 in the development of these structures.
KW - Differential distribution
KW - Fibrillin-1
KW - Fibrillin-2
KW - Human development
KW - Organogenesis
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U2 - 10.1016/S0945-053X(02)00100-2
DO - 10.1016/S0945-053X(02)00100-2
M3 - Article
C2 - 12524050
AN - SCOPUS:0036921311
SN - 0945-053X
VL - 21
SP - 637
EP - 646
JO - Matrix Biology
JF - Matrix Biology
IS - 8
ER -