Abstract:
:Multiple hereditary exostoses (MHE) is an autosomal dominant disorder that affects about 1 in 50,000 children worldwide. MHE, also known as hereditary multiple exostoses (HME) or multiple osteochondromas (MO), is characterized by cartilage-capped outgrowths called osteochondromas that develop adjacent to the growth plates of skeletal elements in young patients. These benign tumors can affect growth plate function, leading to skeletal growth retardation, or deformations, and can encroach on nerves, tendons, muscles, and other surrounding tissues and cause motion impairment, chronic pain, and early onset osteoarthritis. In about 2-5% of patients, the osteochondromas can become malignant and life threatening. Current treatments consist of surgical removal of the most symptomatic tumors and correction of the major skeletal defects, but physical difficulties and chronic pain usually continue and patients may undergo multiple surgeries throughout life. Thus, there is an urgent need to find new treatments to prevent or reverse osteochondroma formation. The 2016 International MHE Research Conference was convened to provide a forum for the presentation of the most up-to-date and advanced clinical and basic science data and insights in MHE and related fields; to stimulate the forging of new perspectives, collaborations, and venues of research; and to publicize key scientific findings within the biomedical research community and share insights and relevant information with MHE patients and their families. This report provides a description, review, and assessment of all the exciting and promising studies presented at the Conference and delineates a general roadmap for future MHE research targets and goals.
journal_name
Connect Tissue Resjournal_title
Connective tissue researchauthors
Phan AQ,Pacifici M,Esko JDdoi
10.1080/03008207.2017.1394295subject
Has Abstractpub_date
2018-01-01 00:00:00pages
85-98issue
1eissn
0300-8207issn
1607-8438journal_volume
59pub_type
杂志文章abstract::This study investigated the in vivo formation of engineering cartilage within living or devitalized cartilage discs using reflectance mode confocal microscopy and conventional light microscopy. Pig articular chondrocytes were suspended in fibrin glue and placed between two cartilage discs. Four experimental groups wer...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200600809935
更新日期:2006-01-01 00:00:00
abstract::Syndecan-4 is emerging as an important player in cell interaction with the extracellular environment and has been shown to be involved in the progression of intervertebral disc degeneration. However, the mechanism of syndecan-4 regulation by TNF-α and the role of TGF-β1 in regulating syndecan-4 expression remain poorl...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2014.996702
更新日期:2015-01-01 00:00:00
abstract::Muscle development in the embryonic chick involves the interfacing of two different cell types: myogenic cells forming the contractile network and connective tissue cells which wrap this network into discrete morphologies. Previous reports from this laboratory detailed the characterization of large extracellular matri...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208809015023
更新日期:1988-01-01 00:00:00
abstract::Although intervertebral disc (IVD) degeneration is one of most common causes of morbidity, its etiology remains unclear. In healthy discs, the rates of synthesis and breakdown of the extracelluar matrix (ECM) are in equilibrium because of intricate regulation by growth factors and catabolic cytokines. Important among ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2013.775258
更新日期:2013-01-01 00:00:00
abstract::Bone sialoprotein (BSP) and osteopontin (OPN) are two extracellular bone matrix proteins that have the ability to modulate the growth of hydroxyapatite in vitro. Studies of BSP/OPN hydroxyapatite interactions in the past have been directed toward the identification of essential structural elements that allow these two...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209609029217
更新日期:1996-01-01 00:00:00
abstract:BACKGROUND:The presence of genetic variants in uric acid (UA) transporters can be associated with hyperuricemia, and therefore with an increased risk of monosodium urate (MSU) crystal precipitation. The inflammatory process triggered by these crystals leads to cartilage damage, which, in turn, could promote knee osteoa...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2018.1483359
更新日期:2019-05-01 00:00:00
abstract::An extensive literature on bone morphogenetic protein (BMP) induced generation and regeneration shows general agreement about one observation. The incidence and quantity of bone were greatest when BMP was delivered with a carrier of various biologic and non biologic polymers. In the present research, neutral lipids ei...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209709160210
更新日期:1997-01-01 00:00:00
abstract::To study the glycosylation of glomerular basement membrane collagen (GBMC) in diabetes, kidneys were obtained at autopsy from 5 patients with insulin-requiring diabetes of long duration and diabetic complications, and from 5 control subjects. Glomeruli were prepared by sieving and collagen was isolated by limited peps...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208209008054
更新日期:1982-01-01 00:00:00
abstract::Experimental lathyrism was induced by feeding newborn chicks a diet containing 0.2 and 0.4% DL-Penicillamine, with or without CuSO4 (10 mg/Kg diet) and Vitamin B6 (100 mg/Kg diet), or 0.015 and 0.1% beta-aminopropionitrile fumarate (beta-APN). After 7, 15, 25 and 55 days of treatment the animals were killed, the aorta...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208509015021
更新日期:1985-01-01 00:00:00
abstract::The purpose of this investigation is to evaluate enamel thickness in extant and extinct hominoids. The material used in this study spans the evolutionary history of this group, from 20 million years ago to the present. The objectives of this investigation are to test three hypotheses: (1) the Loading Hypothesis: loadi...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209809023927
更新日期:1998-01-01 00:00:00
abstract::In the absence of ascorbic acid, confluent human skin fibroblasts incubated in 0.5% serum-supplemented medium had one-third of the level of lysyl hydroxylase activity of cells incubated in media containing high serum concentrations (5-20%). This difference appeared to be due to a decline in the enzyme activity followi...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208509152396
更新日期:1985-01-01 00:00:00
abstract::??Although injuries to the medial collateral ligament (MCL) can heal functionally without surgical intervention, the collagen fibers in the healing tissue remain compromised. The molecular basis for this poor healing potential was investigated by examining extracellular matrix-modifying molecules such as bone morphoge...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2010.524719
更新日期:2011-01-01 00:00:00
abstract::Tubulointerstitial nephritis antigen (TIN-ag) is a recently described basement membrane component reactive with autoantibodies in some forms of autoimmune mediated tubulointerstitial nephritis. Immunofluorescence studies using polyclonal and monoclonal antibodies have indicated a restrictive tissue distribution for TI...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209709160222
更新日期:1997-01-01 00:00:00
abstract::Muscle-derived cells can differentiate into chondrogenic cells. In our present study, we investigated the pattern of expression of Sox9, a transcription factor known to play a key role in chondrogenesis, in a rat myoblastic cell line, L6. In addition, we evaluated expression of type II collagen and myogenic regulatory...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200490514130
更新日期:2004-01-01 00:00:00
abstract::We demonstrated the cells producing collagenase and the time course of collagenase-production at early stages of wound healing, using histology and two immunohistochemical procedures on cross sections of rat skin harvested 0, 3, 5, 7 and 12 days after full-thickness incisions. A monospecific rabbit polyclonal antibody...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209109006995
更新日期:1991-01-01 00:00:00
abstract::Two overlapping clones encompassing 8.5 kb of the human elastin gene were isolated from two genomic libraries constructed by partial digestion with either HaeIII/AluI or Sau3A and contained in lambda Charon 4A or EMBL3, respectively. The 6 kb of DNA comprising the most 3' portion of the gene were sequenced demonstrati...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208709006976
更新日期:1987-01-01 00:00:00
abstract::Oral mandibular epithelium determines the position and number of tooth organs. Cranial neural crest-derived ectomesenchyme provides inductive signals for (i) tooth shape, and (ii) sequential determination and expression of several different epithelial-derived enamel proteins. Inductive signalling follows a precise seq...
journal_title:Connective tissue research
pub_type: 杂志文章,评审
doi:10.3109/03008208909023878
更新日期:1989-01-01 00:00:00
abstract::The effects of various avocado oils on collagen metabolism in skin were studied in growing rats fed diets containing 10% (w/w) of the tested oils. Rats fed the unrefined avocado oil extracted with hexane from the intact fruit, its unsaponifiables or the avocado seed oil, showed significant increases in soluble collage...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209109152159
更新日期:1991-01-01 00:00:00
abstract::A partially purified BMP preparation was combined with a fibrous glass membrane (FGM) or porous particles of hydroxyapatite (PPHAP), and then implanted subcutaneously into the backs of rats. As a control of these new carriers, a conventional carrier of insoluble bone matrix (IBM) was also used. These new geometrically...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209509013726
更新日期:1995-01-01 00:00:00
abstract::Elastic ear cartilage and aortae from nine-day-old hamsters were incubated in Krebs-Ringer medium containing ascorbate, beta-aminopropionitrile and either [14C]proline or [3H]valine. The [14C]proline-labeled proteins were separated by chromatography on Agarose A-5 m in sodium dodecyl sulfate and their [14C]-hydroxypro...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208009152117
更新日期:1980-01-01 00:00:00
abstract:: Purpose/Aim of the study mice are the most often used pre-clinical lab models for studying the pathologies of bone mineralization. However, recent evidence suggests that two of the most often used mice strains (C57BL/6J and CD-1) might show differences in the bone minerali...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008207.2019.1601184
更新日期:2020-09-01 00:00:00
abstract::The effects of two forms of elastase on the Young's Modulus of elastic tissue have been studied. The monomeric, calcium poor form of the enzyme lowers the value of the Young's Modulus before an appreciable amount of protein is taken into solution. The polymeric, calcium rich enzyme on the other hand degrades the prote...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207609152217
更新日期:1976-01-01 00:00:00
abstract::Oriented fibers, arising from the self assembly of collagen, may be produced from in vitro fibrillogenesis of soluble collagen with gradients of fibrillogenic potential. These gradients may be introduced by either concentration gradients of pronase-treated collagen or of arginine which, in turn, create concentration g...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208808992792
更新日期:1988-01-01 00:00:00
abstract::The use of electron microscopic observations to characterize the state of aggregation of collagen molecules in solution has been examined from the point of view that the observed structures must be compatible with the physical chemical properties of the solution. The common procedure of addition of droplets of a colla...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207809152630
更新日期:1978-01-01 00:00:00
abstract:PURPOSE:Orthodontic tooth movement occurs during the bone remodeling induced by therapeutic mechanical strain. It is important to investigate the relation between the strength of mechanical stress and bone formation activity. The aim of this study was to determine the effect of high-magnitude mechanical strain on bone ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2015.1040878
更新日期:2015-01-01 00:00:00
abstract:UNLABELLED:Keratocystic odontogenic tumors (KCOTs) are locally invasive, rapidly proliferating cystic lesions of the jaw. The bone-invasive nature of these tumors has been previously associated with the expression of matrix metalloproteinases (MMPs), which degrade the extracellular matrix. The purpose of this study was...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008207.2014.923875
更新日期:2014-08-01 00:00:00
abstract::The resorption of devitalized bone by isolated osteoclasts in culture was studied by scanning electron microscopy. Osteoclasts attached to the bone and resorbed mineral but left most of matrix collagen undigested in the resorption pits. Postculture-treatment of bone substrate with collagenase completely removed the ma...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008208909023885
更新日期:1989-01-01 00:00:00
abstract::Tobacco smoking is considered a major risk factor for the development and progression of periodontal diseases (Haber, J. and Wattles, J. (1994). J. Periodontol., 64, 16-23). The purpose of this study was to determine the effects of nicotine on rat gingival fibroblasts (RGF) cultured in vitro. After ether anesthesia, r...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008200009005643
更新日期:2000-01-01 00:00:00
abstract::Solutions of type I acid soluble collagen were studied in light and electron microscopy at concentrations over 40 mg/ml. Banded patterns spontaneously emerge in samples observed between crossed polars between slide and coverslip. The textures are interpreted as precholesteric, appearing at the transition between the i...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.3109/03008209809002438
更新日期:1998-01-01 00:00:00
abstract::Raman spectra have been determined for hyaluronan, chondroitin-4-sulfate, chondroitin-6-sulfate, aggrecan monomers and aggregates. The nature of the saccharides and the pattern of sulfation can be discerned. There were only small spectral changes with pH and ionic composition. Differences between hydroxyl vibrations, ...
journal_title:Connective tissue research
pub_type: 杂志文章
doi:10.1080/03008200802398422
更新日期:2009-01-01 00:00:00