Recombinant Osteoprotegerin for Juvenile Paget's Disease
Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin, a key regulator of osteoclastogenesis. The authors investigated the effects of recombinant osteoprotegerin in two adult siblings...
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| Veröffentlicht in: | The New England journal of medicine 2005-09, Vol.353 (9), p.918-923 |
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| creator | Cundy, Tim Davidson, James Rutland, Michael D Stewart, Carolyn DePaoli, Alex M |
| description | Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin, a key regulator of osteoclastogenesis. The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. After 15 months, radial bone mass had increased, skeletal bisphosphonate retention had decreased, and there was radiographic improvement. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The change in the size and shape of the skeleton during growth and its renewal during adult life depend on the closely coordinated activity of bone-resorbing cells (osteoclasts) and bone-forming cells (osteoblasts). The receptor activator of nuclear factor-κB (RANK) ligand (RANKL)–osteoprotegerin–RANK system constitutes a critical mechanism for signaling between osteoblasts and osteoclasts.
1
In response to factors such as parathyroid hormone that stimulate the remodeling cycle, the cytokine RANKL is expressed and secreted by osteoblasts. The interaction of RANKL with RANK, located on preosteoclasts, stimulates the differentiation of these cells into active, bone-resorbing osteoclasts. In parallel with the production of RANKL, . . . |
| doi_str_mv | 10.1056/NEJMoa050893 |
| format | Article |
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The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The change in the size and shape of the skeleton during growth and its renewal during adult life depend on the closely coordinated activity of bone-resorbing cells (osteoclasts) and bone-forming cells (osteoblasts). The receptor activator of nuclear factor-κB (RANK) ligand (RANKL)–osteoprotegerin–RANK system constitutes a critical mechanism for signaling between osteoblasts and osteoclasts.
1
In response to factors such as parathyroid hormone that stimulate the remodeling cycle, the cytokine RANKL is expressed and secreted by osteoblasts. The interaction of RANKL with RANK, located on preosteoclasts, stimulates the differentiation of these cells into active, bone-resorbing osteoclasts. In parallel with the production of RANKL, . . .</description><identifier>ISSN: 0028-4793</identifier><identifier>EISSN: 1533-4406</identifier><identifier>DOI: 10.1056/NEJMoa050893</identifier><identifier>PMID: 16135836</identifier><identifier>CODEN: NEJMAG</identifier><language>eng</language><publisher>Boston, MA: Massachusetts Medical Society</publisher><subject>Adult ; Antibodies - blood ; Biological and medical sciences ; Biomarkers - blood ; Bone Density - drug effects ; Bone Resorption - drug therapy ; Bones ; Disease ; Female ; General aspects ; Glycoproteins - adverse effects ; Glycoproteins - immunology ; Glycoproteins - therapeutic use ; Humans ; Humerus - diagnostic imaging ; Male ; Medical sciences ; Medical treatment ; Mutation ; Osteitis Deformans - drug therapy ; Osteitis Deformans - genetics ; Osteoprotegerin ; Radiography ; Radius - diagnostic imaging ; Radius - drug effects ; Receptors, Cytoplasmic and Nuclear - immunology ; Receptors, Cytoplasmic and Nuclear - therapeutic use ; Receptors, Tumor Necrosis Factor - immunology ; Receptors, Tumor Necrosis Factor - therapeutic use ; Recombinant Proteins - adverse effects ; Recombinant Proteins - therapeutic use ; Siblings ; Side effects</subject><ispartof>The New England journal of medicine, 2005-09, Vol.353 (9), p.918-923</ispartof><rights>Copyright © 2005 Massachusetts Medical Society. All rights reserved.</rights><rights>2005 INIST-CNRS</rights><rights>Copyright 2005 Massachusetts Medical Society.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4463-aaab36894a4a0bd8a2a13534a3faa24dacff68f0e2a6182d5c91d520391331533</citedby><cites>FETCH-LOGICAL-c4463-aaab36894a4a0bd8a2a13534a3faa24dacff68f0e2a6182d5c91d520391331533</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.nejm.org/doi/pdf/10.1056/NEJMoa050893$$EPDF$$P50$$Gmms$$H</linktopdf><linktohtml>$$Uhttps://www.nejm.org/doi/full/10.1056/NEJMoa050893$$EHTML$$P50$$Gmms$$H</linktohtml><link.rule.ids>314,776,780,2746,2747,26080,27901,27902,52357,54039</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=17065918$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16135836$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Cundy, Tim</creatorcontrib><creatorcontrib>Davidson, James</creatorcontrib><creatorcontrib>Rutland, Michael D</creatorcontrib><creatorcontrib>Stewart, Carolyn</creatorcontrib><creatorcontrib>DePaoli, Alex M</creatorcontrib><title>Recombinant Osteoprotegerin for Juvenile Paget's Disease</title><title>The New England journal of medicine</title><addtitle>N Engl J Med</addtitle><description>Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin, a key regulator of osteoclastogenesis. The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. After 15 months, radial bone mass had increased, skeletal bisphosphonate retention had decreased, and there was radiographic improvement. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The change in the size and shape of the skeleton during growth and its renewal during adult life depend on the closely coordinated activity of bone-resorbing cells (osteoclasts) and bone-forming cells (osteoblasts). The receptor activator of nuclear factor-κB (RANK) ligand (RANKL)–osteoprotegerin–RANK system constitutes a critical mechanism for signaling between osteoblasts and osteoclasts.
1
In response to factors such as parathyroid hormone that stimulate the remodeling cycle, the cytokine RANKL is expressed and secreted by osteoblasts. The interaction of RANKL with RANK, located on preosteoclasts, stimulates the differentiation of these cells into active, bone-resorbing osteoclasts. In parallel with the production of RANKL, . . .</description><subject>Adult</subject><subject>Antibodies - blood</subject><subject>Biological and medical sciences</subject><subject>Biomarkers - blood</subject><subject>Bone Density - drug effects</subject><subject>Bone Resorption - drug therapy</subject><subject>Bones</subject><subject>Disease</subject><subject>Female</subject><subject>General aspects</subject><subject>Glycoproteins - adverse effects</subject><subject>Glycoproteins - immunology</subject><subject>Glycoproteins - therapeutic use</subject><subject>Humans</subject><subject>Humerus - diagnostic imaging</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Medical treatment</subject><subject>Mutation</subject><subject>Osteitis Deformans - drug therapy</subject><subject>Osteitis Deformans - genetics</subject><subject>Osteoprotegerin</subject><subject>Radiography</subject><subject>Radius - diagnostic imaging</subject><subject>Radius - drug effects</subject><subject>Receptors, Cytoplasmic and Nuclear - 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Academic</collection><jtitle>The New England journal of medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Cundy, Tim</au><au>Davidson, James</au><au>Rutland, Michael D</au><au>Stewart, Carolyn</au><au>DePaoli, Alex M</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Recombinant Osteoprotegerin for Juvenile Paget's Disease</atitle><jtitle>The New England journal of medicine</jtitle><addtitle>N Engl J Med</addtitle><date>2005-09-01</date><risdate>2005</risdate><volume>353</volume><issue>9</issue><spage>918</spage><epage>923</epage><pages>918-923</pages><issn>0028-4793</issn><eissn>1533-4406</eissn><coden>NEJMAG</coden><abstract>Juvenile Paget's disease, a genetic bone disease characterized by accelerated bone turnover, results from inactivating mutations in the gene encoding osteoprotegerin, a key regulator of osteoclastogenesis. The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. After 15 months, radial bone mass had increased, skeletal bisphosphonate retention had decreased, and there was radiographic improvement. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The authors investigated the effects of recombinant osteoprotegerin in two adult siblings with juvenile Paget's disease. Osteoprotegerin may be therapeutic in juvenile Paget's disease.
The change in the size and shape of the skeleton during growth and its renewal during adult life depend on the closely coordinated activity of bone-resorbing cells (osteoclasts) and bone-forming cells (osteoblasts). The receptor activator of nuclear factor-κB (RANK) ligand (RANKL)–osteoprotegerin–RANK system constitutes a critical mechanism for signaling between osteoblasts and osteoclasts.
1
In response to factors such as parathyroid hormone that stimulate the remodeling cycle, the cytokine RANKL is expressed and secreted by osteoblasts. The interaction of RANKL with RANK, located on preosteoclasts, stimulates the differentiation of these cells into active, bone-resorbing osteoclasts. In parallel with the production of RANKL, . . .</abstract><cop>Boston, MA</cop><pub>Massachusetts Medical Society</pub><pmid>16135836</pmid><doi>10.1056/NEJMoa050893</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adult Antibodies - blood Biological and medical sciences Biomarkers - blood Bone Density - drug effects Bone Resorption - drug therapy Bones Disease Female General aspects Glycoproteins - adverse effects Glycoproteins - immunology Glycoproteins - therapeutic use Humans Humerus - diagnostic imaging Male Medical sciences Medical treatment Mutation Osteitis Deformans - drug therapy Osteitis Deformans - genetics Osteoprotegerin Radiography Radius - diagnostic imaging Radius - drug effects Receptors, Cytoplasmic and Nuclear - immunology Receptors, Cytoplasmic and Nuclear - therapeutic use Receptors, Tumor Necrosis Factor - immunology Receptors, Tumor Necrosis Factor - therapeutic use Recombinant Proteins - adverse effects Recombinant Proteins - therapeutic use Siblings Side effects |
| title | Recombinant Osteoprotegerin for Juvenile Paget's Disease |
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