Advances in the pathogenesis and possible treatments for multiple hereditary exostoses from the 2016 international MHE conference.

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 Res

authors

Phan AQ,Pacifici M,Esko JD

doi

10.1080/03008207.2017.1394295

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

85-98

issue

1

eissn

0300-8207

issn

1607-8438

journal_volume

59

pub_type

杂志文章
  • Tissue engineered cartilage integration to live and devitalized cartilage: a study by reflectance mode confocal microscopy and standard histology.

    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

    authors: Peretti GM,Campo-Ruiz V,Gonzalez S,Randolph MA,Wei Xu J,Morse KR,Roses RE,Yaremchuk MJ

    更新日期:2006-01-01 00:00:00

  • TNF-α and TGF-β1 regulate Syndecan-4 expression in nucleus pulposus cells: role of the mitogen-activated protein kinase and NF-κB pathways.

    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

    authors: Yang H,Liu H,Li X,Pan H,Li Z,Wang J,Zheng Z

    更新日期:2015-01-01 00:00:00

  • Initial characterization of small proteoglycans synthesized by embryonic chick leg muscle-associated connective tissues.

    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

    authors: Young HE,Carrino DA,Caplan AI

    更新日期:1988-01-01 00:00:00

  • Synergistic effect of combined growth factors in porcine intervertebral disc degeneration.

    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

    authors: Cho H,Lee S,Park SH,Huang J,Hasty KA,Kim SJ

    更新日期:2013-01-01 00:00:00

  • Generation and use of recombinant human bone sialoprotein and osteopontin for hydroxyapatite studies.

    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

    authors: Stubbs JT 3rd

    更新日期:1996-01-01 00:00:00

  • Common gene variants interactions related to uric acid transport are associated with knee osteoarthritis susceptibility.

    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

    authors: Fernández-Torres J,Martínez-Nava GA,Oliviero F,López-Reyes AG,Martínez-Flores K,Garrido-Rodríguez D,Francisco-Balderas A,Zamudio-Cuevas Y

    更新日期:2019-05-01 00:00:00

  • Lipids closely associated with bone morphogenetic protein (BMP)--and induced heterotopic bone formation. With preliminary observations of deficiencies in lipid and osteoinduction in lathyrism in rats.

    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

    authors: Urist MR,Behnam K,Kerendi F,Raskin K,Nuygen TD,Shamie AN,Malinin TI

    更新日期:1997-01-01 00:00:00

  • Glycosylation of human glomerular basement membrane collagen: increased content of hexose in ketoamine linkage and unaltered hydroxylysine-O-glycosides in patients with diabetes.

    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

    authors: Uitto J,Perejda AJ,Grant GA,Rowold EA,Kilo C,Williamson JR

    更新日期:1982-01-01 00:00:00

  • Alterations of elastin fibrogenesis by inhibition of the formation of desmosine crosslinks. Comparison between the effect of beta-aminopropionitrile (beta-APN) and penicillamine.

    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

    authors: Ronchetti IP,Contri MB,Fornieri C,Quaglino D Jr,Mori G

    更新日期:1985-01-01 00:00:00

  • Evolutionary and functional significance of hominoid tooth enamel.

    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

    authors: Gantt DG,Rafter JA

    更新日期:1998-01-01 00:00:00

  • Serum stimulation of lysyl hydroxylase activity in cultured human skin fibroblasts.

    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

    authors: Murad S,Sivarajah A,Pinnell SR

    更新日期:1985-01-01 00:00:00

  • Molecular events surrounding collagen fibril assembly in the early healing rabbit medial collateral ligament--failure to recapitulate normal ligament development.

    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

    authors: Achari Y,Chin JW,Heard BJ,Rattner JB,Shrive NG,Frank CB,Hart DA

    更新日期:2011-01-01 00:00:00

  • Detection of tubulointerstitial nephritis antigen (TIN-ag) in Lewis rat.

    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

    authors: Nelson TR,Butkowski RJ,Michael AF,Charonis AS

    更新日期:1997-01-01 00:00:00

  • Expression of transcription factor Sox9 in rat L6 myoblastic cells.

    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

    authors: Matsushita T,Matsui N,Fujioka H,Kubo S,Kuroda R,Kurosaka M,Yoshiya S

    更新日期:2004-01-01 00:00:00

  • Collagenase production at the border of granulation tissue in a healing wound: macrophage and mesenchymal collagenase production in vivo.

    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

    authors: Porras-Reyes BH,Blair HC,Jeffrey JJ,Mustoe TA

    更新日期:1991-01-01 00:00:00

  • Structure of the 3' region of the human elastin gene: great abundance of Alu repetitive sequences and few coding sequences.

    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

    authors: Indik Z,Yoon K,Morrow SD,Cicila G,Rosenbloom J,Rosenbloom J,Ornstein-Goldstein N

    更新日期:1987-01-01 00:00:00

  • Positional signalling and patterning for amelogenesis in mouse molar tooth development.

    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

    authors: Slavkin HC

    更新日期:1989-01-01 00:00:00

  • The effect of various avocado oils on skin collagen metabolism.

    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

    authors: Werman MJ,Mokady S,Nimni ME,Neeman I

    更新日期:1991-01-01 00:00:00

  • Two distinctive BMP-carriers induce zonal chondrogenesis and membranous ossification, respectively; geometrical factors of matrices for cell-differentiation.

    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

    authors: Kuboki Y,Saito T,Murata M,Takita H,Mizuno M,Inoue M,Nagai N,Poole AR

    更新日期:1995-01-01 00:00:00

  • Biosynthesis of tropoelastin by elastic cartilage.

    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

    authors: Heeger P,Rosenbloom J

    更新日期:1980-01-01 00:00:00

  • Raman spectroscopy investigation shows that mineral maturity is greater in CD-1 than in C57BL/6 mice distal femurs after sexual maturity.

    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

    authors: Fayolle C,Labrune M,Berteau JP

    更新日期:2020-09-01 00:00:00

  • The physical properties of elastic fibers and elastolysis.

    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

    authors: Hall DA

    更新日期:1976-01-01 00:00:00

  • Oriented fibrillogenesis of collagen in vitro by ordered convection.

    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

    authors: Ghosh S,Comper WD

    更新日期:1988-01-01 00:00:00

  • The visualization of individual collagen molecules and aggregates in collagen solution: the effect of preparation and sampling techniques.

    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

    authors: Schwartz D,Veis A

    更新日期:1978-01-01 00:00:00

  • High-magnitude mechanical strain inhibits the differentiation of bone-forming rat calvarial progenitor cells.

    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

    authors: Fushiki R,Mayahara K,Ogawa M,Takahashi Y,Karasawa Y,Tsurumachi N,Tamura T,Shimizu N

    更新日期:2015-01-01 00:00:00

  • Matrix metalloproteinase expression in keratocystic odontogenic tumors and primary cells.

    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

    authors: Amm HM,Casimir MD,Clark DB,Sohn P,MacDougall M

    更新日期:2014-08-01 00:00:00

  • Matrix collagen of devitalized bone is resistant to osteoclastic bone resorption.

    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

    authors: Shimizu H,Sakamoto S,Sakamoto M

    更新日期:1989-01-01 00:00:00

  • Effect of nicotine on rat gingival fibroblasts in vitro.

    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

    authors: Lahmouzi J,Simain-Sato F,Defresne MP,De Pauw MC,Heinen E,Grisar T,Legros JJ,Legrand R

    更新日期:2000-01-01 00:00:00

  • Banded patterns in liquid crystalline phases of type I collagen: relationship with crimp morphology in connective tissue architecture.

    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

    authors: Giraud-Guille MM,Besseau L

    更新日期:1998-01-01 00:00:00

  • Structural analysis of glycosaminoglycans and proteoglycans by means of Raman microspectrometry.

    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

    authors: Ellis R,Green E,Winlove CP

    更新日期:2009-01-01 00:00:00