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Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXVII | Pages 19 - 19
1 Jun 2012
Yu J Li B Fairbank J Urban J
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Introduction

Elastic fibres are constructed of a central core of elastin surrounded by microfibrils that are composed mainly of fibrillin-1 and fibrillin-2. Patients with mutations in the gene encoding fibrillin-1 or fibrillin-2 develop Marfan syndrome or Beals syndrome (congenital contractural arachnodactyly), respectively. Scoliosis is one of the clinical manifestations in these patients, but how a defect in the elastic proteins could lead to a spinal deformity is not clear. On the one hand, the mutations could induce scoliosis via mechanical means as they could lead to alterations in the biomechanics of the elastic fibre system. On the other hand, elastic fibres also bind growth factors such as transforming growth factor β (TGFβ) and bone morphogenic proteins (BMPs), and the mutations could hence change patterns of spinal growth.

Methods

We have investigated the localisation of elastic proteins in different spinal tissues at different stages of curve development in mouse models and in human tissue obtained during scoliosis surgery.