Fibrillin-3 expression in human development

Laetitia Sabatier, Nicolai Miosge, Dirk Hubmacher, Guoqing Lin, Elaine C. Davis, Dieter P. Reinhardt

Research output: Contribution to journalArticlepeer-review

54 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)43-52
Number of pages10
JournalMatrix Biology
Volume30
Issue number1
DOIs
StatePublished - Jan 2011
Externally publishedYes

Keywords

  • Basement membranes
  • Connective tissue
  • Development
  • Fibrillin
  • Immunohistochemistry
  • Microfibrils

Fingerprint

Dive into the research topics of 'Fibrillin-3 expression in human development'. Together they form a unique fingerprint.

Cite this