학술논문

Co-expression of FBN1 with mesenchyme-specific genes in mouse cell lines: implications for phenotypic variability in Marfan syndrome.
Document Type
Article
Source
European Journal of Human Genetics. Nov2010, Vol. 18 Issue 11, p1209-1215. 7p. 2 Charts, 1 Graph.
Subject
*MESENCHYME
*MARFAN syndrome
*CONNECTIVE tissue diseases
*GENETIC disorders
*MUSCLE cells
Language
ISSN
1018-4813
Abstract
Mutations in the human FBN1 gene cause Marfan syndrome, a complex disease affecting connective tissues but with a highly variable phenotype. To identify genes that might participate in epistatic interactions with FBN1, and could therefore explain the observed phenotypic variability, we have looked for genes that are co-expressed with Fbn1 in the mouse. Microarray expression data derived from a range of primary mouse cells and cell lines were analysed using the network analysis tool BioLayout Express3D. A cluster of 205 genes, including Fbn1, were selectively expressed by mouse cell lines of different mesenchymal lineages and by mouse primary mesenchymal cells (preadipocytes, myoblasts, fibroblasts, osteoblasts). Promoter analysis of this gene set identified several candidate transcriptional regulators. Genes within this co-expressed cluster are candidate genetic modifiers for Marfan syndrome and for other connective tissue diseases. [ABSTRACT FROM AUTHOR]