Abstract：
:Several rare inherited disorders have been described that show phenotypic overlap with Paget's disease of bone (PDB) and in which PDB is a component of a multisystem disorder affecting muscle and the central nervous system. These conditions are the subject of this review article. Insertion mutations within exon 1 of the TNFRSF11A gene, encoding the receptor activator of nuclear factor kappa B (RANK), cause severe PDB-like disorders including familial expansile osteolysis, early-onset familial PDB and expansile skeletal hyperphosphatasia. The mutations interfere with normal processing of RANK and cause osteoclast activation through activation of nuclear factor kappa B (NFκB) independent of RANK ligand stimulation. Recessive, loss-of-function mutations in the TNFRSF11B gene, which encodes osteoprotegerin, cause juvenile PDB and here the bone disease is due to unopposed activation of RANK by RANKL. Multisystem proteinopathy is a disorder characterised by myopathy and neurodegeneration in which PDB is often an integral component. It may be caused by mutations in several genes including VCP, HNRNPA1, HNRNPA2B1, SQSTM1, MATR3, and TIA1, some of which are involved in classical PDB. The mechanisms of osteoclast activation in these conditions are less clear but may involve NFκB activation through sequestration of IκB. The evidence base for management of these disorders is somewhat limited due to the fact they are extremely rare. Bisphosphonates have been successfully used to gain control of elevated bone remodelling but as yet, no effective treatment exists for the treatment of the muscle and neurological manifestations of MSP syndromes.
journal_name
Calcif Tissue Intjournal_title
Calcified tissue internationalauthors
Ralston SH,Taylor JPdoi
10.1007/s00223-019-00520-5subject
Has Abstractpub_date
2019-05-01 00:00:00pages
501-516issue
5eissn
0171-967Xissn
1432-0827pii
10.1007/s00223-019-00520-5journal_volume
104pub_type
杂志文章,评审abstract：:Cytochemical staining of normal human bone cells in monolayer cultures for alkaline phosphatase (ALP) indicated that the cultures contained mixed-cell populations. Time course evaluations of the cytochemical staining revealed, in addition to the ALP-negative cell population, at least two subpopulations of ALP-positive...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02555909
更新日期：1990-11-01 00:00:00
abstract：:There are few studies of the effect of smoking on bone density in young women. The reported antiestrogenic effect of smoking could be a mechanism for a possible effect of smoking on bone. We measured bone mineral density (BMD) by dual-energy X-ray absorptiometry (whole body, proximal femur, lumbar spine), and serum le...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF00301626
更新日期：1992-04-01 00:00:00
abstract：:Skeletal tissues contain, apart from cells of the osteogenic and chondrogenic lineage, cells of hemopoietic origin, e.g., macrophages, osteoclasts, and their precursors. In the present study we examined the sensitivity for extracellular ATP4- of the above-mentioned cell types in freshly isolated, bone-derived cell pop...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF00297190
更新日期：1994-08-01 00:00:00
abstract：:Alkaline phosphatase is detected in both soluble and particulate fractions of rat bone. The concentration of alkaline phosphatase in the particulate fraction of rat skull cap bone and femur is high during the peak period of calcification of these bones suggesting the possibility of using it as a marker for the rate of...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02555116
更新日期：1986-09-01 00:00:00
abstract：:Several phosphoprotein preparations (phosvitin, rat incisor and fetal calf molar dentin phosphoproteins) all inhibit apatite growth/replication from pre-existing crystal seeds in metastable solutions. Two stages of the crystal growth process were inhibited by these phosphoproteins. First an initial lag period was indu...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02407188
更新日期：1980-01-01 00:00:00
abstract：:Hepatitis C treatment with interferon alpha-2b (IFN-alpha) and ribavirin has been related to decreased bone mineral density. The aim of this study was to investigate the in vitro effects of different concentrations of ribavirin and IFN-alpha on osteoblast-like cells. Human osteoblast-like cells obtained by the outgrow...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-004-0175-0
更新日期：2004-08-01 00:00:00
abstract：:Test implant plates surgically retrieved from distal femurs of dogs were studied by Raman spectroscopy in order to characterize the bone-implant interface. The implant surface consisted of phosphate mineral, plasma sprayed on a titanium substrate. On the basis of its spectroscopic signature, the phosphate mineral of b...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02556157
更新日期：1991-05-01 00:00:00
abstract：:Mechanical forces applied to cultured bone cells induce the production of cAMP via stimulation of the formation of prostaglandin E2 (PGE2) and its release into the medium, resulting in stimulation of adenylate cyclase. In this paper we show that either the antibiotic gentamycin (100 micrograms/ml) or antiphospholipid ...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02553753
更新日期：1988-04-01 00:00:00
abstract：:Bone mineral content (BMC) and bone mineral density (BMD) are common but imperfect surrogate measures of bone strength. The mechanical response tissue analyzer is a device that measures long bone bending stiffness (EI), which strongly predicts bone breaking strength. We hypothesized that isokinetic resistance training...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-009-9247-5
更新日期：2009-06-01 00:00:00
abstract：:The purpose of this study was to investigate the mineral induction capacity in vitro of polyanionic proteins covalently bound to a surface. Rat dentin gamma-carboxyglutamate-containing protein of the osteocalcin type (Gla-protein), proteoglycan (PG), and phosphoprotein (PP-H), as well as phosvitin (PhV) and bovine ser...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02553763
更新日期：1989-04-01 00:00:00
abstract：:Proton pump inhibitors are taken by millions of patients for prevention and treatment of gastroesophageal diseases. Case-control studies have suggested that use of omeprazole is associated with an increased risk of hip fractures. The aim of this prospective study was to assess the risk of vertebral fractures in postme...
journal_title：Calcified tissue international
pub_type： 杂志文章,多中心研究
doi：10.1007/s00223-008-9188-4
更新日期：2009-01-01 00:00:00
abstract：:Sustained exposure to high levels of parathyroid hormone (PTH), as observed in hyperparathyroidism, is catabolic to bone. The increase in the RANKL/OPG ratio in response to continuous PTH, resulting in increased osteoclastogenesis, is well established. However, the effects of prolonged PTH exposure on key regulators o...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-016-0176-9
更新日期：2016-11-01 00:00:00
abstract：:1,25(OH)2D3 was found to regulate its own receptor levels via an increase in corresponding mRNA levels in human osteoblast-like osteosarcoma cells (MG-63). In addition, exposure of the cells for 24h to dexamethasone, estradiol, retinoic acid, or triiodothyronine resulted in a dose-dependent accumulation of hVDR mRNA. ...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02555101
更新日期：1991-01-01 00:00:00
abstract：:The present study reports on the separation of fluid from soft, "cheeselike" enamel of porcine permanent teeth in the secretory stage, and the determination of its chemical composition. The enamel tissues were dissected from mandibles of 5 to 6-month-old piglets and pooled under mineral oil in centrifuge tubes, and th...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02555250
更新日期：1987-08-01 00:00:00
abstract：:Vitamin D supplements are often recommended to restore sufficiency, although the adherence to treatment is low. This study assessed vitamin D status at different time intervals following the cessation of treatment. The database of Clalit-Health-Services (CHS), a not-for-profit HMO covering more than half of the Israel...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-011-9536-7
更新日期：2011-11-01 00:00:00
abstract：:The goal of this study was to find out if bone can recover after long-term administration of bisphosphonate. Disodium pamidronate (APD) was given orally by gavage to mature beagle dogs at doses of 0, 2.5, 12.5, and 25 mg/kg/day for 1 year (0.1% concentration) and the animals were allowed to recover for another year. A...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF00310408
更新日期：1994-10-01 00:00:00
abstract：:Bone and mineral metabolism has been reported to affect the development of the ossification of the posterior longitudinal ligament (OPLL). The aim of this study was to compare bone mineral densities (BMD) and rate of osteoporosis between cervical OPLL and a matched control group. We also investigated the correlation o...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-012-9662-x
更新日期：2013-01-01 00:00:00
abstract：:Extracellular ATP, and to a lesser extent adenosine, an ATP metabolite, stimulated cell proliferation in osteoblast-like cells (MC3T3-E1). ATP increased cytosolic Ca2+ due to Ca2+ mobilization from intracellular storage in the same concentration range of the nucleotide as that effective for DNA synthesis, suggesting t...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02529732
更新日期：1996-02-01 00:00:00
abstract：:Methotrexate (MTX) is one of the most commonly prescribed drugs for autoimmune rheumatic diseases. As there is no consensus on its negative effects on bone, the purpose of this investigation was to determine the clinical spectrum of patients with stress fractures due to long-term MTX treatment (i.e., MTX osteopathy). ...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-020-00765-5
更新日期：2021-02-01 00:00:00
abstract：:Further insight into human tooth enamel, dense fraction (TE), has been obtained by following the change and loss of CO3(2-), OH-, structurally incorporated H2O, Cl-, and, indirectly, HPO4(2-) after TE had been heated in N2 or vacuum in the range 25-1000 degrees C. Quantitative infrared spectroscopic, lattice parameter...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02407181
更新日期：1980-01-01 00:00:00
abstract：:The drawbacks of estrogen restrict the clinical use of hormone replacement therapy, and it would be most helpful to explore new estrogenic substances that could prevent bone loss and be free from any adverse effects. We synthesized a new compound named bone-seeking estrogen (SE2) by combining 17β-estradiol (E2) with i...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-013-9739-1
更新日期：2013-08-01 00:00:00
abstract：:ELISAs for measuring the urinary excretion of collagen crosslinks and related peptides appear to show marked differences in sensitivity to anti-resorptive therapy. This presumably reflects variations in specificity of the anylate being detected in these assays, and the way in which they respond to treatment. To clarif...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s002239900148
更新日期：1996-11-01 00:00:00
abstract：:The aim of this work was to explore the reduction of fluoride concentrations in the skeleton after stopping experimental fluoride administration. Fluoride was administered to the rats at varying doses (0, 50, 100 ppm in drinking water) and for different lengths of time (4, 13, 25 weeks). A series of fluoride concentra...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF00318049
更新日期：1995-04-01 00:00:00
abstract：:The potassium pyroantimonate technique was utilized for the selective subcellular localization of calcium in the mandibular condylar cartilage of 1-day-old rats. Electron dense calcium pyroantimonate precipitates were localized principally in mitochondria and at the cell membrane of the chondrocytes. In addition, smal...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02408603
更新日期：1980-01-01 00:00:00
abstract：:Prosected vertebrae and phalanges from 32 cadavera were examined using noninvasive methods commonly used for assessment of osteoporosis. Bone measures were then compared with a strength variable obtained by mechanically crushing or breaking the bones. All of the phalangeal density assessment techniques were found to b...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02555150
更新日期：1988-08-01 00:00:00
abstract：:KH1060 is a new 20-epi-vitamin D3 analog, which has exerted a considerable immunosuppressive potency in vitro. We have tested in vivo the effect of KH1060 on the suppression of renal allograft rejection in the rat. Allogenic kidney transplantation from DA donor rats to Lewis recipient rats treated intraperitoneally wi...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF00296066
更新日期：1994-02-01 00:00:00
abstract：:Insulin is a potent stimulator of collagen synthesis and other osteoblastic cell functions. In various insulin-sensitive tissues, stimulation of glucose transport and glycolytic metabolism are hallmarks of insulin action and may play a role in insulin regulation of cellular function. However, the effects of insulin on...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/BF02556657
更新日期：1989-07-01 00:00:00
abstract：:Newly developed fuzzy logic-derived structural parameters were used to characterize trabecular bone architecture in high-resolution magnetic resonance imaging (HR-MRI) of human cadaver calcaneus specimens. These parameters were compared to standard histomorphological structural measures and analyzed concerning perform...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-007-9058-5
更新日期：2007-10-01 00:00:00
abstract：:This study sought to develop a noninvasive, reliable, clinically relevant, and easy-to-implement mouse model that can be used for investigation of the pathophysiology of PTOA and for preclinical testing of new therapies of PTOA. Accordingly, we have established a closed intraarticular tibial plateau compression loadin...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-019-00614-0
更新日期：2020-02-01 00:00:00
abstract：:We describe here the activity of a novel selective estrogen receptor modulator, SP500263. When given to adult ovariectomized (OVX) rats for 28 days at doses of 0.3, 1, or 3 mg/kg/day, we found that SP500263 partially protected against OVX-induced loss of bone mineral content in the distal ends of femurs and in the who...
journal_title：Calcified tissue international
pub_type： 杂志文章
doi：10.1007/s00223-002-1029-2
更新日期：2003-06-01 00:00:00
