ArticleHuman molecular genetics2014
Induced ablation of Bmp1 and Tll1 produces osteogenesis imperfecta in mice.
Article in Human molecular genetics, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 68 citations in OpenAlex.
- Pathogenesis and precision management of osteogenesis imperfecta: from genetic mechanisms to novel therapies.World journal of pediatrics : WJP · 2026Review
- Murine model of high bone mass osteogenesis imperfecta exhibits bone matrix hyper-mineralization, misaligned mineral crystals, and altered osteoblast differentiation.Bone research · 2026Article
- New knock-in mouse linesJBMR plus · 2026Article
- SLIT3 fragments orchestrate neurovascular expansion and thermogenesis in brown adipose tissue.Nature communications · 2026Article
- Elevated serum BMP1 in pubertal female rats and girls with central precocious puberty: a potential biomarker for longitudinal growth.Endocrine connections · 2025Article
- Osteocyte Dendrites: How Do They Grow, Mature, and Degenerate in Mineralized Bone?Cytoskeleton (Hoboken, N.J.) · 2025Review
- Alendronate treatment rescues the effects of compressive loading of TMJ in osteogenesis imperfecta mice.Progress in orthodontics · 2024Article
- Preclinical Rodent Models for Human Bone Disease, Including a Focus on Cortical Bone.Endocrine reviews · 2024Review
- Expression quantitative trait loci analysis in rheumatoid arthritis identifies tissue specific variants associated with severity and outcome.Annals of the rheumatic diseases · 2024Article
- Identification of PCPE-2 as the endogenous specific inhibitor of human BMP-1/tolloid-like proteinases.Nature communications · 2023Article
- Activin receptor-like kinase 3: a critical modulator of development and function of mineralized tissues.Frontiers in cell and developmental biology · 2023Review
- Pathways Controlling Formation and Maintenance of the Osteocyte Dendrite Network.Current osteoporosis reports · 2022Review
- Sexually dimorphic estrogen sensing in skeletal stem cells controls skeletal regeneration.Nature communications · 2022Article
- An Update on Animal Models of Osteogenesis Imperfecta.Calcified tissue international · 2022Review
- Gene Expression Profiles of Human Mesenchymal Stromal Cells Derived from Wharton's Jelly and Amniotic Membrane before and after Osteo-Induction Using NanoString Platform.Current issues in molecular biology · 2022Article
- Between a rock and a hard place: Regulation of mineralization in the periodontium.Genesis (New York, N.Y. : 2000) · 2022Review
- BMP1 is not required for lung fibrosis in mice.Scientific reports · 2022Article
- Osteogenesis Imperfecta: Mechanisms and Signaling Pathways Connecting Classical and Rare OI Types.Endocrine reviews · 2022Review
- Procollagen C-proteinase enhancer-1 (PCPE-1), a potential biomarker and therapeutic target for fibrosis.Matrix biology plus · 2021Article
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Authors and funding
10 authors at 6 institutions in 1 country.
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Abstract
Osteogenesis imperfecta (OI), or brittle bone disease, is most often caused by dominant mutations in the collagen I genes COL1A1/COL1A2, whereas rarer recessive OI is often caused by mutations in genes encoding collagen I-interacting proteins. Recently, mutations in the gene for the proteinase bone morphogenetic 1 (BMP1) were reported in two recessive OI families. BMP1 and the closely related proteinase mammalian tolloid-like 1 (mTLL1) are co-expressed in various tissues, including bone, and have overlapping activities that include biosynthetic processing of procollagen precursors into mature collagen monomers. However, early lethality of Bmp1- and Tll1-null mice has precluded use of such models for careful study of in vivo roles of their protein products. Here we employ novel mouse strains with floxed Bmp1 and Tll1 alleles to induce postnatal, simultaneous ablation of the two genes, thus avoiding barriers of Bmp1(-/-) and Tll1(-/-) lethality and issues of functional redundancy. Bones of the conditionally null mice are dramatically weakened and brittle, with spontaneous fractures-defining features of OI. Additional skeletal features include osteomalacia, thinned/porous cortical bone, reduced processing of procollagen and dentin matrix protein 1, remarkably high bone turnover and defective osteocyte maturation that is accompanied by decreased expression of the osteocyte marker and Wnt-signaling inhibitor sclerostin, and by marked induction of canonical Wnt signaling. The novel animal model presented here provides new opportunities for in-depth analyses of in vivo roles of BMP1-like proteinases in bone and other tissues, and for their roles, and for possible therapeutic interventions, in OI.
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