Stem cell research to help fight brittle-bone disease osteogenesis imperfecta

A study involving Professor Raymond Dalgleish (pictured) from the Department of Genetics is to be conducted for the first time involving the transplantation of stem cells into foetuses with the brittle-bone disease osteogenesis imperfecta (OI), which causes repeated fractures, often before birth.

Children born with severe forms of OI, or congenital brittle bone disease, are often seriously disabled, with the most severe sub-type resulting in death shortly after birth. Repeated fractures in all parts of the skeleton, especially the long bones, give rise to physical disabilities, postural aberration, and stunted growth.

Collagen is a thread-like protein found in bone that has a similar reinforcing function to iron rods in concrete. Since the disease is caused by an inability of the developing body to form normal collagen, scientists at Karolinska Institutet have produced a special strain of stem cell which, when injected into the body of sufferers, targets and strengthens the bone by producing collagen.

Professor Dalgleish said: “My role in the project will be to advise on the genetic data collection and analysis for the transplanted stem cells and for the recipient patients. This work builds on more than thirty years’ experience which I have amassed in the genetics of osteogenesis imperfecta. This is an exciting project with the potential to provide much improved quality of life for children affected by severe forms of this disorder.”

The “Boost Brittle Bones Before Birth” (BOOSTB4) project will start in early 2016 and will be coordinated by Karolinska Institutet.

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