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Reports from Goteborg University describe recent advances in life sciences
[April 05, 2007]

Reports from Goteborg University describe recent advances in life sciences


(Science Letter Via Thomson Dialog NewsEdge)
A new study, "Filamin B deficiency in mice results in skeletal malformations and impaired microvascular development," is now available. "Mutations in filamin B (FLNB), a gene encoding a cytoplasmic actin-binding protein, have been found in human skeletal disorders, including boomerang dysplasia, spondylocarpotarsal syndrome, Larsen syndrome, and atelosteogenesis phenotypes I and III. To examine the role of FLNB in vivo, we generated mice with a targeted disruption of Flnb," scientists in Sweden report.



"Fewer than 3% of homozygous embryos reached term, indicating that Flnb is important in embryonic development. Heterozygous mutant mice were indistinguishable from their wild-type siblings. Flnb was ubiquitously expressed; strong expression was found in endothelial cells and chondrocytes. Flnb-deficient fibroblasts exhibited more disorganized formation of actin filaments and reduced ability to migrate compared with wild-type controls. Flnb-deficient embryos exhibited impaired development of the microvasculature and skeletal system. The few Flnb-deficient mice that were born were very small and had severe skeletal malformations, including scoliotic and kyphotic spines, lack of intervertebral discs, fusion of vertebral bodies, and reduced hyaline matrix in extremities, thorax, and vertebrae. These mice died or had to be euthanized before 4 weeks of age," wrote X. Zhou and colleagues, Goteborg University.

The researchers concluded: "Thus, the phenotypes of Flnb-deficient mice closely resemble those of human skeletal disorders with mutations in FLNB."


Zhou and colleagues published their study in Proceedings of the National Academy of Sciences of the United States of America (Filamin B deficiency in mice results in skeletal malformations and impaired microvascular development. Proceedings of the National Academy of Sciences of the United States of America, 2007;104(10):3919-24).

For additional information, contact X. Zhou, Sahlgrenska Center for Cardiovascular and Metabolic Research, Wallenberg Laboratory, Goteborg University, SE 413 45 Goteborg, Sweden.

The publisher's contact information for the journal Proceedings of the National Academy of Sciences of the United States of America is: National Acad Sciences, 2101 Constitution Avenue NW, Washington, DC 20418, USA.

Keywords: Sweden, Life Sciences.

This article was prepared by Science Letter editors from staff and other reports. Copyright 2007, Science Letter via NewsRx.com.

Copyright 2007 Science Letter via NewsRx.com

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