Fibrodysplasia ossificans progressiva (FOP), a disorder of ectopic osteogenesis, misregulates cell surface expression and trafficking of BMPRIA.
about
A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressivaOveractive bone morphogenetic protein signaling in heterotopic ossification and Duchenne muscular dystrophyClassic and atypical fibrodysplasia ossificans progressiva (FOP) phenotypes are caused by mutations in the bone morphogenetic protein (BMP) type I receptor ACVR1From mysteries to medicines: drug development for fibrodysplasia ossificans progressiveEarly mortality and cardiorespiratory failure in patients with fibrodysplasia ossificans progressiva.Evolutionary mutant models for human diseaseCharacterization of dystrophic calcification induced in mice by cardiotoxinMolecular consequences of the ACVR1(R206H) mutation of fibrodysplasia ossificans progressiva.The FOP metamorphogene encodes a novel type I receptor that dysregulates BMP signaling.Progesterone receptor activates Msx2 expression by downregulating TNAP/Akp2 and activating the Bmp pathway in EpH4 mouse mammary epithelial cells.The revolution in human monogenic disease mapping.Molecular, phenotypic aspects and therapeutic horizons of rare genetic bone disorders.Fibrodysplasia ossificans progressiva: mechanisms and models of skeletal metamorphosisInsights from a rare genetic disorder of extra-skeletal bone formation, fibrodysplasia ossificans progressiva (FOP)Alk2 regulates early chondrogenic fate in fibrodysplasia ossificans progressiva heterotopic endochondral ossificationFibrodysplasia ossificans progressiva: a human genetic disorder of extraskeletal bone formation, or--how does one tissue become another?Role of altered signal transduction in heterotopic ossification and fibrodysplasia ossificans progressiva.Proximal tibial osteochondromas in patients with fibrodysplasia ossificans progressiva.Dysregulation of the BMP-4 signaling pathway in fibrodysplasia ossificans progressiva.Inherited human diseases of heterotopic bone formation.Skeletal metamorphosis in fibrodysplasia ossificans progressiva (FOP)When one skeleton is enough: approaches and strategies for the treatment of fibrodysplasia ossificans progressiva (FOP).A new era for fibrodysplasia ossificans progressiva: a druggable target for the second skeleton.Granting immunity to FOP and catching heterotopic ossification in the Act.Regulation of chondrogenesis and chondrocyte differentiation by stress.Fibrodysplasia ossificans progressivaDysregulated BMP signaling and enhanced osteogenic differentiation of connective tissue progenitor cells from patients with fibrodysplasia ossificans progressiva (FOP).The ACVR1 R206H mutation found in fibrodysplasia ossificans progressiva increases human induced pluripotent stem cell-derived endothelial cell formation and collagen production through BMP-mediated SMAD1/5/8 signalingThe fibrodysplasia ossificans progressiva R206H ACVR1 mutation activates BMP-independent chondrogenesis and zebrafish embryo ventralization.Osteogenic gene expression correlates with development of heterotopic ossification in war wounds.Fibrodysplasia ossificans progressiva: diagnosis, management, and therapeutic horizonsInvestigations of activated ACVR1/ALK2, a bone morphogenetic protein type I receptor, that causes fibrodysplasia ossificans progressiva.In vitro analyses of the dysregulated R206H ALK2 kinase-FKBP12 interaction associated with heterotopic ossification in FOP.Bone morphogenetic protein-5, a key molecule that mediates differentiation in MC3T3E1 osteoblast cell line.TGF-β Family Signaling in Connective Tissue and Skeletal Diseases.Cellular Hypoxia Promotes Heterotopic Ossification by Amplifying BMP Signaling.New Protocol to Optimize iPS Cells for Genome Analysis of Fibrodysplasia Ossificans Progressiva.Variable signaling activity by FOP ACVR1 mutations.The Horizon of a Therapy for Rare Genetic Diseases: A "Druggable" Future for Fibrodysplasia Ossificans Progressiva.
P2860
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P2860
Fibrodysplasia ossificans progressiva (FOP), a disorder of ectopic osteogenesis, misregulates cell surface expression and trafficking of BMPRIA.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年学术文章
@wuu
2005年学术文章
@zh
2005年学术文章
@zh-cn
2005年学术文章
@zh-hans
2005年学术文章
@zh-my
2005年学术文章
@zh-sg
2005年學術文章
@yue
2005年學術文章
@zh-hant
name
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@en
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@nl
type
label
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@en
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@nl
prefLabel
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@en
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@nl
P2093
P2860
P356
P1476
Fibrodysplasia ossificans prog ...... ion and trafficking of BMPRIA.
@en
P2093
Eileen M Shore
Frederick S Kaplan
Jennifer L Fiori
Lourdes Serrano de la Peña
Paul C Billings
P2860
P304
P356
10.1359/JBMR.050305
P577
2005-03-07T00:00:00Z