Bone Abstracts

Introduction: Duchenne muscular dystrophy (DMD) is caused by mutation in the dystrophin gene on the X-chromosome, leading to progressive deterioration in muscle function from early childhood. Corticosteroid treatment prolongs the time to loss of walking ability and improves life span. The combination of muscular weakness, reduced mobility and steroids increases the risk of secondary osteoporosis.Subjects and methods: In this prospective observational stu...

Caspases belong to cysteine proteases participating in apoptosis and inflammation. However, there is recent evidence about their functions also in other events such as cell differentiation. This is supported also by our latest research of odontogenesis and osteogenesis. With the increasing interest in caspases due to their apoptosis-related therapies but also emerging non-apoptotic roles, exact evaluation of their impact at single cell level becomes challenging.<p class="a...

The c-Myb transcription factor is associated with proliferation of undifferentiated cells in number of tissues, but recent data suggests its role also in differentiation. c-Myb is important in formation of the cartilage, bone and apparently also in hard tissue mineralization (Matalova et al. 2011).Embryonic micromasses were established from mouse front limbs at the embryonic day E12. Micromass cultures represent an effective tool for experimenta...

Caspases are the basic enzymatic components in the apoptotic process. Recently, these molecules seem to participate not only in apoptotic but also in other cellular events, particularly in differentiation. This work aimed to further investigate novel roles of executive caspases such as caspase-7. The investigation is based on our previously published findings showing non-apoptotic roles of caspase-7 in odontogenesis.In this study, we followed the localiz...

Osteogenesis Imperfecta (OI) is in most cases a congenital disease of collagen. The mutations have been reported in COLIA1 and COLIA2 genes, localised to chromosomes 17 and 7 respectively. The incidence at birth is 6–20/100 000. Children and youths with OI often display a complex and heterogeneous picture with fragile skeleton, fractures, curvature in the long bones, short stature, pain and limitations in mobility and everyday activity. The impact of those factors for the...

Introduction: Osteogenesis imperfecta (OI) is a genetic disorder which mainly affects the collagen in the bone mass with fractures and deformities as the main symptoms. In OI there is a great variation in dysfunction related to the disease. Mobility and activities related to mobility are often most difficult. The objective for this study was to find a relevant, valid and reliable instrument to assess the children’s activity capability.Method and par...

Objectives: Craniosynostosis is a skeletal birth defect, resulting in premature fusion of cranial sutures. The patency of the sutures is essential to enable the growth of the skull in compliance to the developing brain. Mutations in TWIST1 and TCF12 have been identified in patients with Saethre-Chotzen syndrome, which is typically associated with coronal synostosis. Studies in mouse models suggest that the quantity of TCF12-TWIST1 heterodimers is one critical...

Caspase-7 belongs to executive apoptotic and pro-inflammatory caspases. Recent reports point to novel roles of these caspases, particularly in cell differentiation. As we revealed such function of caspase-7 in odontogenesis, we turned our attention to participation of this molecule in formation of hard tissue in general.In this work, both type of bones, intramembranous (mandibular/alveolar bone), and endochondral (long bones) were investigated. Mouse hea...

Among activation of apoptotic machinery, Fas (CD95)/FasL (CD178) were suggested to act in cell proliferation and differentiation. Expression of Fas and FasL was reported during tooth and bone formation. The examination of gld mice showed increased total body bone mass and number of osteoblasts in long bones. However, Fas and FasL functions in osteogenesis remain controversial. As most of studies dealing with Fas/FasL system in bone formation were performed in endochondral mode...

There is a similarity of the mechanisms of physiological and pathological mineralization. Pathological calcification of soft tissues as the case of vascular calcification is characterized by the deposition of hydroxyapaptite induced by matrix vesicles at least in the initial stage as in the case of physiological calcification in skeletal tissues. Lipid metabolism is involved in the differentiation of smooth muscle cells and bone cells suggesting that phospholipases can modulat...