Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development

Fabio Quondamatteo, Dieter P. Reinhardt, Noe L. Charbonneau, Gabriele Pophal, Lynn Sakai, Rainer Herken

Research output: Contribution to journalArticle

58 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)637-646
Number of pages10
JournalMatrix Biology
Volume21
Issue number8
DOIs
StatePublished - Dec 1 2002
Externally publishedYes

Fingerprint

Embryonic and Fetal Development
Microfibrils
Notochord
Kidney
Lung
Marfan Syndrome
Organogenesis
Organ Culture Techniques
Ribs
Fibrillin-2
Fibrillin-1
Ganglia
Connective Tissue
Aorta
Glycoproteins
Fetus
Central Nervous System
Monoclonal Antibodies
Skin
Mutation

Keywords

  • Differential distribution
  • Fibrillin-1
  • Fibrillin-2
  • Human development
  • Organogenesis

ASJC Scopus subject areas

  • Molecular Biology

Cite this

Quondamatteo, F., Reinhardt, D. P., Charbonneau, N. L., Pophal, G., Sakai, L., & Herken, R. (2002). Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biology, 21(8), 637-646. https://doi.org/10.1016/S0945-053X(02)00100-2

Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. / Quondamatteo, Fabio; Reinhardt, Dieter P.; Charbonneau, Noe L.; Pophal, Gabriele; Sakai, Lynn; Herken, Rainer.

In: Matrix Biology, Vol. 21, No. 8, 01.12.2002, p. 637-646.

Research output: Contribution to journalArticle

Quondamatteo, F, Reinhardt, DP, Charbonneau, NL, Pophal, G, Sakai, L & Herken, R 2002, 'Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development', Matrix Biology, vol. 21, no. 8, pp. 637-646. https://doi.org/10.1016/S0945-053X(02)00100-2
Quondamatteo F, Reinhardt DP, Charbonneau NL, Pophal G, Sakai L, Herken R. Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biology. 2002 Dec 1;21(8):637-646. https://doi.org/10.1016/S0945-053X(02)00100-2
Quondamatteo, Fabio ; Reinhardt, Dieter P. ; Charbonneau, Noe L. ; Pophal, Gabriele ; Sakai, Lynn ; Herken, Rainer. / Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. In: Matrix Biology. 2002 ; Vol. 21, No. 8. pp. 637-646.
@article{e3fe10b1926548f7beb8e3b6001a34be,
title = "Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development",
abstract = "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.",
keywords = "Differential distribution, Fibrillin-1, Fibrillin-2, Human development, Organogenesis",
author = "Fabio Quondamatteo and Reinhardt, {Dieter P.} and Charbonneau, {Noe L.} and Gabriele Pophal and Lynn Sakai and Rainer Herken",
year = "2002",
month = "12",
day = "1",
doi = "10.1016/S0945-053X(02)00100-2",
language = "English (US)",
volume = "21",
pages = "637--646",
journal = "Matrix Biology",
issn = "0945-053X",
publisher = "Elsevier",
number = "8",

}

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

AU - Herken, Rainer

PY - 2002/12/1

Y1 - 2002/12/1

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

UR - http://www.scopus.com/inward/record.url?scp=0036921311&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036921311&partnerID=8YFLogxK

U2 - 10.1016/S0945-053X(02)00100-2

DO - 10.1016/S0945-053X(02)00100-2

M3 - Article

VL - 21

SP - 637

EP - 646

JO - Matrix Biology

JF - Matrix Biology

SN - 0945-053X

IS - 8

ER -