title: Fine mapping of the human biglycan (BGN) gene within the Xq28 region employing a hybrid cell panel.
abstract: Human biglycan is a small proteoglycan that is expressed at high levels in the growing skeleton and in human skin at the cell surface of differentiating keratinocytes. The human gene for biglycan (BGN) has previously been mapped by in situ hybridization to the Xq27-q28 region. Employing somatic hybrid cell lines with human X chromosome breakpoints within this region, we performed a fine mapping of the gene within Xq28. Our results indicate that the biglycan gene is proximal to the red/green cone pigment genes, G6PD, and coagulation factor VIII and is distal to DXS304, DXS305, and GABRA3. The biglycan gene precisely maps to a region of the X chromosome, where, by comparative gene mapping, one would expect to find the gene for X-linked dominant chondrodysplasia punctata/ichthyosis/short stature (Happle) syndrome. Hence, BGN is a candidate gene for the Happle syndrome.
authors.affiliation: Department of Human Genetics, University Hospital, Nijmegen, The Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: Traupe
authors.forename: A M
authors.initials: AM
authors.lastname: van den Ouweland
authors.forename: B A
authors.initials: BA
authors.lastname: van Oost
authors.forename: W
authors.initials: W
authors.lastname: Vogel
authors.forename: U
authors.initials: U
authors.lastname: Vetter
authors.forename: S T
authors.initials: ST
authors.lastname: Warren
authors.forename: M
authors.initials: M
authors.lastname: Rocchi
authors.forename: M G
authors.initials: MG
authors.lastname: Darlison
authors.forename: H H
authors.initials: HH
authors.lastname: Ropers
mesh_terms: D000818:Animals; D001483:Base Sequence; D058578:Biglycan; D002386:Cataract / Q000235:genetics; D002806:Chondrodysplasia Punctata / Q000235:genetics; D002874:Chromosome Mapping; D006224:Cricetinae; D004392:Dwarfism / Q000235:genetics; D016326:Extracellular Matrix Proteins; D005796:Genes; D005819:Genetic Markers; D006801:Humans; D006822:Hybrid Cells; D016114:Ichthyosis, X-Linked / Q000235:genetics; D051379:Mice; D008969:Molecular Sequence Data; D011509:Proteoglycans / Q000235:genetics*; D013577:Syndrome; D014960:X Chromosome
chemical_list: C546884:BGN protein, human; C546885:Bgn protein, mouse; D058578:Biglycan; D016326:Extracellular Matrix Proteins; D005819:Genetic Markers; D011509:Proteoglycans