TY - JOUR
T1 - Fibrillin-3 expression in human development
AU - Sabatier, Laetitia
AU - Miosge, Nicolai
AU - Hubmacher, Dirk
AU - Lin, Guoqing
AU - Davis, Elaine C.
AU - Reinhardt, Dieter P.
N1 - Funding Information:
We thank Dr. Lynn Sakai (Shriners Hospital for Children, Portland, OR) for generously providing the F2 and the 143 monoclonal antibodies. This work was supported by the Canadian Institutes of Health Research grants MOP-68836 , MOP-106494 and IMH-102821 and the Natural Sciences and Engineering Research Council of Canada grant RGPIN 375738-09 .
PY - 2011/1
Y1 - 2011/1
N2 - Fibrillin proteins are the major components of extracellular microfibrils found in many connective tissues. Fibrillin-1 and fibrillin-2 are well studied and mutations in these proteins cause a number of fibrillinopathies including Marfan syndrome and congenital contractural arachnodactyly, respectively. Fibrillin-3 was more recently discovered and is much less well characterized. Fibrillin-1 is expressed throughout life, whereas fibrillins-2 and -3 are thought to be primarily present during development. Here, we report detailed fibrillin-3 expression patterns in early human development. A polyclonal antiserum against a C-terminal recombinant half of human fibrillin-3 was produced in rabbit. Anti-fibrillin-3 antibodies were affinity-purified and antibodies cross-reacting with the other fibrillins were removed by absorption resulting in specific anti-fibrillin-3 antibodies. Immunohistochemical analyses with these purified antibodies demonstrate that fibrillin-3 is temporally expressed in numerous tissues relatively evenly from the 6th to the 12th gestational week. Fibrillin-3 was found spatially expressed in perichondrium, perineurium, perimysium, skin, developing bronchi, glomeruli, pancreas, kidney, heart and testis and at the prospective basement membranes in developing epithelia and endothelia. Double immunohistochemical analyses showed that all fibrillins are globally expressed in the same organs, with a number of differences on the tissue level in cartilage, perichondrium and developing bronchi. These results suggest that fibrillin-3, compared to the other fibrillins, fulfills both overlapping and distinct functions in human development.
AB - Fibrillin proteins are the major components of extracellular microfibrils found in many connective tissues. Fibrillin-1 and fibrillin-2 are well studied and mutations in these proteins cause a number of fibrillinopathies including Marfan syndrome and congenital contractural arachnodactyly, respectively. Fibrillin-3 was more recently discovered and is much less well characterized. Fibrillin-1 is expressed throughout life, whereas fibrillins-2 and -3 are thought to be primarily present during development. Here, we report detailed fibrillin-3 expression patterns in early human development. A polyclonal antiserum against a C-terminal recombinant half of human fibrillin-3 was produced in rabbit. Anti-fibrillin-3 antibodies were affinity-purified and antibodies cross-reacting with the other fibrillins were removed by absorption resulting in specific anti-fibrillin-3 antibodies. Immunohistochemical analyses with these purified antibodies demonstrate that fibrillin-3 is temporally expressed in numerous tissues relatively evenly from the 6th to the 12th gestational week. Fibrillin-3 was found spatially expressed in perichondrium, perineurium, perimysium, skin, developing bronchi, glomeruli, pancreas, kidney, heart and testis and at the prospective basement membranes in developing epithelia and endothelia. Double immunohistochemical analyses showed that all fibrillins are globally expressed in the same organs, with a number of differences on the tissue level in cartilage, perichondrium and developing bronchi. These results suggest that fibrillin-3, compared to the other fibrillins, fulfills both overlapping and distinct functions in human development.
KW - Basement membranes
KW - Connective tissue
KW - Development
KW - Fibrillin
KW - Immunohistochemistry
KW - Microfibrils
UR - http://www.scopus.com/inward/record.url?scp=78951480069&partnerID=8YFLogxK
U2 - 10.1016/j.matbio.2010.10.003
DO - 10.1016/j.matbio.2010.10.003
M3 - Article
C2 - 20970500
AN - SCOPUS:78951480069
SN - 0945-053X
VL - 30
SP - 43
EP - 52
JO - Matrix Biology
JF - Matrix Biology
IS - 1
ER -