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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
4
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pubmed:dateCreated |
2003-4-3
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pubmed:abstractText |
Fibrodysplasia ossificans progressiva is a rare genetic disorder characterized by congenital malformations of the great toes and by progressive heterotopic bone formation. Bone morphogenetic protein-4 (BMP-4) messenger ribonucleic acid (mRNA) and protein are uniquely overexpressed in lymphocytes and lesional cells from patients who have fibrodysplasia ossificans progressiva. However, the BMP-4 gene is not mutated in fibrodysplasia ossificans progressiva. The activities of BMPs are specified in part by the formation of morphogen gradients that are further regulated by an array of secreted antagonists. Recent studies have indicated that BMP-4 upregulates the expression of the BMP antagonists noggin, gremlin, and follistatin, thereby establishing an autoregulatory feedback loop. Therefore, a defect in the feedback pathway between BMP-4 and one or more of its extracellular antagonists could contribute to the elevated BMP-4 activity characteristic of fibrodysplasia ossificans progressiva.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/BMP4 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Bone Morphogenetic Protein 4,
http://linkedlifedata.com/resource/pubmed/chemical/Bone Morphogenetic Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Carrier Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Follistatin,
http://linkedlifedata.com/resource/pubmed/chemical/GREM1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Glycoproteins,
http://linkedlifedata.com/resource/pubmed/chemical/Intercellular Signaling Peptides...,
http://linkedlifedata.com/resource/pubmed/chemical/Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/RNA, Messenger,
http://linkedlifedata.com/resource/pubmed/chemical/chordin,
http://linkedlifedata.com/resource/pubmed/chemical/noggin protein
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pubmed:status |
MEDLINE
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pubmed:month |
Apr
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pubmed:issn |
0021-9355
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
85-A
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
667-74
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pubmed:dateRevised |
2010-10-25
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pubmed:meshHeading |
pubmed-meshheading:12672843-Bone Morphogenetic Protein 4,
pubmed-meshheading:12672843-Bone Morphogenetic Proteins,
pubmed-meshheading:12672843-Carrier Proteins,
pubmed-meshheading:12672843-Cell Line,
pubmed-meshheading:12672843-Feedback, Physiological,
pubmed-meshheading:12672843-Follistatin,
pubmed-meshheading:12672843-Gene Expression,
pubmed-meshheading:12672843-Glycoproteins,
pubmed-meshheading:12672843-Humans,
pubmed-meshheading:12672843-Intercellular Signaling Peptides and Proteins,
pubmed-meshheading:12672843-Myositis Ossificans,
pubmed-meshheading:12672843-Ossification, Heterotopic,
pubmed-meshheading:12672843-Proteins,
pubmed-meshheading:12672843-RNA, Messenger
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pubmed:year |
2003
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pubmed:articleTitle |
Paresis of a bone morphogenetic protein-antagonist response in a genetic disorder of heterotopic skeletogenesis.
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pubmed:affiliation |
University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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