Hunter syndrome (mucopolysaccharidosis type 11, or MPS 11) results from a deficiency of iduronate-2-sulfatase (IDS) activity due to a primary genetic defect in the X-chromosomal iduronate-2-sulfatase gene. We have studied a 10-year-old male, diagnosed with Hunter syndrome at age 2 years, who underwe
A comparative biochemical analysis of glycosaminoglycans and proteoglycans in human orthotopic and heterotopic bone
β Scribed by Vassiliki M. Mania; Alkiviadis G. Kallivokas; Christina Malavaki; Athanasia P. Asimakopoulou; John Kanakis; Achilleas D. Theocharis; George Klironomos; George Gatzounis; Athanasia Mouzaki; Elias Panagiotopoulos; Nikos K. Karamanos
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 166 KB
- Volume
- 61
- Category
- Article
- ISSN
- 1521-6543
- DOI
- 10.1002/iub.167
No coin nor oath required. For personal study only.
β¦ Synopsis
Abstract
Glycosaminoglycans and proteoglycans are macromolecules of the bone and are involved in the assembly, maturation, mineralization, and maintenance of the extracellular matrix. Heterotopic ossification is the rapid development of calcified bone tissue at ectopic sites of the body, mainly in soft tissues that normally do not ossify. The aim of this study was to characterize the molecular profiles of glycosaminoglycans and proteoglycans in normal and heterotopic bone samples to assess whether differences exist between orthotopic and heterotopic bone. Heterotopic bone tissues contained lower amounts of glycosaminoglycans compared to normal femoral bone. Structural analysis of chondroitin sulfate (CS) revealed that both heterotopic and normal femoral bones were composed mainly of 6βsulfated disaccharides. Quantitative differences in the disaccharide composition of CS, such as the decrease of 6βsulfated disaccharides in heterotopic bone with a concurrent increase of 4βsulfated and nonsulfated disaccharides, were found between ectopic bone and normal femoral bone. The proteoglycans decorin and aggrecan were both detected in all bone samples using specific antibodies. The detection of minor amounts of aggrecan in mature human bone, such as femoral bone, as well as in ectopic bone is described for the first time. These results may elucidate the phenomenon of ectopic bone formation and assist in early detection. Β© 2009 IUBMB IUBMB Life, 61(4): 447β452, 2009
π SIMILAR VOLUMES
## Abstract ## Objective To investigate the longitudinal changes both in the urinary concentrations of biochemical markers and in bone mineral density (BMD) during disease progression in the STR/Ort mouse model of osteoarthritis (OA). ## Methods Male STR/Ort mice were studied, with CBA mice used
Activin A, a member of the TGF-β€ superfamily, plays roles in differentiation and development, including hematopoiesis. Our previous studies indicated that the expression of activin A by human bone marrow cells and monocytes is highly regulated by inflammatory cytokines and glucocorticoids. The prese
A detailed histopathologic analysis of 399 primary breast carcinomas was performed, and several morphologic features were correlated with the estrogen receptor (ER) status. In all cases ER status was determined immunocytochemically by estrogen receptor immunocytochemical assay (ER-ICA). In 359 carci