Associated Papers
Inhibition of FGFR signaling with infigratinib improves linear bone growth in the female Aga2/+ mouse model of osteogenesis imperfecta.
Kot A et al.
Jbmr Plus, 2026
Dissecting primary versus secondary effects of osteogenesis imperfecta on abnormal lung development and function.
Dimori M et al.
Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research, 2026
Modeling rare genetic skeletal disorders with bone organoids: a narrative review.
Micha D et al.
Bone, 2026
A New Perspective on Osteogenesis Imperfecta: From Cellular Mechanisms to the Systemic Impact of Collagen Dysfunction.
Lugli E et al.
International Journal of Molecular Sciences, 2026
LC3 and GABARAP independent autophagy of misfolded procollagen in mouse osteoblasts.
Makareeva E et al.
Autophagy, 2025
Nanoscale Structural and Functional Impacts of Disease-Associated Collagen Mutations.
Tobita CA et al.
Biorxiv : the Preprint Server for Biology, 2025
Anti-transforming growth factor-β treatment shows increased bone mass and strength in a novel mouse model for osteogenesis imperfecta type I.
Busschers E et al.
Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research, 2025
Osteogenesis imperfecta: exploring an autoimmune and immunotherapy perspective.
Goddard JF et al.
Jbmr Plus, 2025
Enhancing Wnt signaling lowers fracture incidence in a severe mouse model of Osteogenesis Imperfecta.
Montagna G et al.
Biorxiv : the Preprint Server for Biology, 2025
Collagen formation, function and role in kidney disease.
De Gregorio V et al.
Nature Reviews. Nephrology, 2025
Characterization of a Novel Col1a1G643S/+ Osteogenesis Imperfecta Mouse Model with Insights into Skeletal Phenotype, Fragility, and Therapeutic Evaluations.
Saitou H et al.
Calcified Tissue International, 2025
Extra-Skeletal Manifestations in Osteogenesis Imperfecta Mouse Models.
Crawford TK et al.
Calcified Tissue International, 2024
Update on the Genetics of Osteogenesis Imperfecta.
Jovanovic M et al.
Calcified Tissue International, 2024
Bone Quality and Mineralization and Effects of Treatment in Osteogenesis Imperfecta.
Misof BM et al.
Calcified Tissue International, 2024
Sestrin2 drives ER-phagy in response to protein misfolding.
De Leonibus C et al.
Developmental Cell, 2024
Unraveling the Link of Altered TGFβ Signaling with Scoliotic Vertebral Malformations in Osteogenesis Imperfecta: A Comprehensive Review.
Kaspiris A et al.
Journal of Clinical Medicine, 2024
Collagen mutation and age contribute to differential craniofacial phenotypes in mouse models of osteogenesis imperfecta.
Sung HH et al.
Jbmr Plus, 2024
Altered Sox9 and FGF signaling gene expression in Aga2 OI mice negatively affects linear growth.
Zieba J et al.
Jci Insight, 2023
Absence of TRIC-B from type XIV Osteogenesis Imperfecta osteoblasts alters cell adhesion and mitochondrial function - A multi-omics study.
Jovanovic M et al.
Matrix Biology : Journal of the International Society for Matrix Biology, 2023
Collagen piezoelectricity in osteogenesis imperfecta and its role in intrafibrillar mineralization.
Kwon J et al.
Communications Biology, 2022
A multi-model approach to predict efficacious clinical dose for an anti-TGF-β antibody (GC2008) in the treatment of osteogenesis imperfecta.
Mavroudis PD et al.
Cpt: Pharmacometrics & Systems Pharmacology, 2022
An Update on Animal Models of Osteogenesis Imperfecta.
Lv F et al.
Calcified Tissue International, 2022
Rosemary Extract-Induced Autophagy and Decrease in Accumulation of Collagen Type I in Osteogenesis Imperfecta Skin Fibroblasts.
Sutkowska-Skolimowska J et al.
International Journal of Molecular Sciences, 2022
Pubertal growth in osteogenesis imperfecta caused by pathogenic variants in COL1A1/COL1A2.
Robinson ME et al.
Genetics in Medicine : Official Journal of the American College of Medical Genetics, 2022
The effect of carbamazepine on bone structure and strength in control and osteogenesis imperfecta (Col1a2 +/p.G610C ) mice.
Blank M et al.
Journal of Cellular and Molecular Medicine, 2022
Dissecting the phenotypic variability of osteogenesis imperfecta.
Garibaldi N et al.
Disease Models & Mechanisms, 2022
Collagen misfolding mutations: the contribution of the unfolded protein response to the molecular pathology.
Bateman JF et al.
Connective Tissue Research, 2022
Antagonism Between PEDF and TGF-β Contributes to Type VI Osteogenesis Imperfecta Bone and Vascular Pathogenesis.
Kang H et al.
Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research, 2022
Targeting TGF-β for treatment of osteogenesis imperfecta.
Song IW et al.
The Journal of Clinical Investigation, 2022
4PBA reduces growth deficiency in osteogenesis imperfecta by enhancing transition of hypertrophic chondrocytes to osteoblasts.
Scheiber AL et al.
Jci Insight, 2022
Osteogenesis Imperfecta: Mechanisms and Signaling Pathways Connecting Classical and Rare OI Types.
Jovanovic M et al.
Endocrine Reviews, 2022
Targeting cellular stress in vitro improves osteoblast homeostasis, matrix collagen content and mineralization in two murine models of osteogenesis imperfecta.
Garibaldi N et al.
Matrix Biology : Journal of the International Society for Matrix Biology, 2021
Morphological and mechanical characterization of bone phenotypes in the Amish G610C murine model of osteogenesis imperfecta.
Kohler R et al.
Plos One, 2021
Signaling pathways affected by mutations causing osteogenesis imperfecta.
Etich J et al.
Cellular Signalling, 2020
Osteogenesis imperfecta: an update on clinical features and therapies.
Marom R et al.
European Journal of Endocrinology, 2020
Genetic Disorders of the Extracellular Matrix.
Lamandé SR et al.
Anatomical Record (hoboken, N.j. : 2007), 2020
Fracture Healing in Collagen-Related Preclinical Models of Osteogenesis Imperfecta.
Zieba J et al.
Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research, 2020
Osteogenesis Imperfecta: New Perspectives From Clinical and Translational Research.
Tauer JT et al.
Jbmr Plus, 2019
Development of a Novel Next-Generation Sequencing Assay for Carrier Screening in Old Order Amish and Mennonite Populations of Pennsylvania.
Crowgey EL et al.
The Journal of Molecular Diagnostics : Jmd, 2019
COL1A2 p.Gly1066Val variant identified in a Han Chinese family with osteogenesis imperfecta type I.
Wang M et al.
Molecular Genetics & Genomic Medicine, 2019