Osteonectin, bone proteoglycan, and phosphophoryn defects in a form of bovine osteogenesis imperfecta.
نویسندگان
چکیده
منابع مشابه
Collagen defects in lethal perinatal osteogenesis imperfecta.
Quantitative and qualitative abnormalities of collagen were observed in tissues and fibroblast cultures from 17 consecutive cases of lethal perinatal osteogenesis imperfecta (OI). The content of type I collagen was reduced in OI dermis and bone and the content of type III collagen was also reduced in the dermis. Normal bone contained 99.3% type I and 0.7% type V collagen whereas OI bone contain...
متن کاملBrittle Bone Disease (Osteogenesis Imperfecta)
Brittle bone disease (Osteogenesis imperfecta first defined by McKusick in 1956, is a disease that causes extremely fragile bones. It is a (OI)), congenital disease meaning that it is present during birth. It is often caused by a defect in the gene that produces type I collagen an important building block of bone and the most abundant protein found in the body. This gene can be affected in many...
متن کاملIntrinsic Modulus of Bone in Osteogenesis Imperfecta – a Nanoindentation Study
INTRODUCTION Osteogenesis imperfecta (OI) is a genetic disorder that affects the production of type I collagen. A major characteristic of OI is bone fragility, caused in part by bone mass deficiency [1]. Impaired collagen network and abnormal mineralization have also been observed in OI [2,3], suggesting that bone material properties are also compromised. Little data, however, is yet available ...
متن کاملUltra-structural defects cause low bone matrix stiffness despite high mineralization in osteogenesis imperfecta mice☆
Bone is a complex material with a hierarchical multi-scale organization from the molecule to the organ scale. The genetic bone disease, osteogenesis imperfecta, is primarily caused by mutations in the collagen type I genes, resulting in bone fragility. Because the basis of the disease is molecular with ramifications at the whole bone level, it provides a platform for investigating the relations...
متن کاملStatic and dynamic bone histomorphometry in children with osteogenesis imperfecta.
Osteogenesis imperfecta (OI) is a genetic disorder characterized by increased bone fragility and low bone mass. Four clinical types are commonly distinguished. Schematically, type I is the mildest phenotype, type II is usually lethal, type III is the most severe form compatible with postnatal survival, and type IV is moderately severe. Although mutations affecting collagen type I are responsibl...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 1984
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.81.7.2213