4PBA reduces growth deficiency in osteogenesis imperfecta by enhancing transition of hypertrophic chondrocytes to osteoblasts

Noncanonical ER–Golgi trafficking and autophagy of endogenous procollagen in osteoblasts

(2021) Laura Gorrell et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Sclerostin-Antibody Treatment Decreases Fracture Rates in Axial Skeleton and Improves the Skeletal Phenotype in Growing oim/oim Mice

(2020) Mickaël Cardinal et al.   CALCIFIED TISSUE INTERNATIONAL

Fracture healing in collagen‐related preclinical models of Osteogenesis Imperfecta

(2020) Jennifer Zieba et al.   JOURNAL OF BONE AND MINERAL RESEARCH

IRX3 and IRX5 inhibit adipogenic differentiation of hypertrophic chondrocytes and promote osteogenesis

(2020) Zhijia Tan et al.   JOURNAL OF BONE AND MINERAL RESEARCH

The extended chondrocyte lineage: implications for skeletal homeostasis and disorders

(2019) Kwok Yeung Tsang et al.   CURRENT OPINION IN CELL BIOLOGY

Defective autophagy in osteoblasts induces endoplasmic reticulum stress and causes remarkable bone loss

(2018) Huixia Li et al.   Autophagy

4-PBA ameliorates cellular homeostasis in fibroblasts from osteogenesis imperfecta patients by enhancing autophagy and stimulating protein secretion

(2018) Roberta Besio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Extracellular vesicles released from mesenchymal stromal cells stimulate bone growth in osteogenesis imperfecta

(2018) Satoru Otsuru et al.   CYTOTHERAPY

Growth characteristics in individuals with osteogenesis imperfecta in North America: results from a multicenter study

(2018) Mahim Jain et al.   GENETICS IN MEDICINE

Carbamazepine reduces disease severity in a mouse model of metaphyseal chondrodysplasia type Schmid caused by a premature stop codon (Y632X) in the Col10a1 gene

(2018) Mitra Forouhan et al.   HUMAN MOLECULAR GENETICS

Substitutions for arginine at position 780 in triple helical domain of the α1(I) chain alter folding of the type I procollagen molecule and cause osteogenesis imperfecta

(2018) Elena Makareeva et al.   PLoS One

Inhibiting the integrated stress response pathway prevents aberrant chondrocyte differentiation thereby alleviating chondrodysplasia

(2018) Cheng Wang et al.   eLife

Longitudinal growth curves for children with classical osteogenesis imperfecta (types III and IV) caused by structural pathogenic variants in type I collagen

(2018) Lauren A. Barber et al.   GENETICS IN MEDICINE

Endoplasmic reticulum stress is induced in growth plate hypertrophic chondrocytes in G610C mouse model of osteogenesis imperfecta

(2018) Amanda L. Scheiber et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Activating the unfolded protein response in osteocytes causes hyperostosis consistent with craniodiaphyseal dysplasia

(2017) Wilson Cheuk Wing Chan et al.   HUMAN MOLECULAR GENETICS

The chaperone activity of 4PBA ameliorates the skeletal phenotype of Chihuahua, a zebrafish model for dominant osteogenesis imperfecta

(2017) Roberta Gioia et al.   HUMAN MOLECULAR GENETICS

Increased intracellular proteolysis reduces disease severity in an ER stress–associated dwarfism

(2017) Lorna A. Mullan et al.   JOURNAL OF CLINICAL INVESTIGATION

Osteogenesis imperfecta

(2017) Joan C. Marini et al.   Nature Reviews Disease Primers

The integrated stress response

(2016) Karolina Pakos‐Zebrucka et al.   EMBO REPORTS

Sclerostin Antibody Treatment Improves the Bone Phenotype ofCrtap-/-Mice, a Model of Recessive Osteogenesis Imperfecta

(2016) Ingo Grafe et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Correlations Between Bone Mechanical Properties and Bone Composition Parameters in Mouse Models of Dominant and Recessive Osteogenesis Imperfecta and the Response to Anti-TGF-β Treatment

(2016) Xiaohong Bi et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Osteoblast Malfunction Caused by Cell Stress Response to Procollagen Misfolding in α2(I)-G610C Mouse Model of Osteogenesis Imperfecta

(2016) Lynn S Mirigian et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Osteogenesis imperfecta

(2016) Antonella Forlino et al.   LANCET

mTORC1 regulates PTHrP to coordinate chondrocyte growth, proliferation and differentiation

(2016) Bo Yan et al.   Nature Communications

Rapidly growing Brtl/+ mouse model of osteogenesis imperfecta improves bone mass and strength with sclerostin antibody treatment

(2015) Benjamin P. Sinder et al.   BONE

Genomic and functional comparison of mesenchymal stromal cells prepared using two isolation methods

(2015) Satoru Otsuru et al.   CYTOTHERAPY

The therapeutic effects of 4-phenylbutyric acid in maintaining proteostasis

(2015) P.S. Kolb et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

ER Stress During the Pubertal Growth Spurt Results in Impaired Long-Bone Growth in Chondrocyte-Specific ERp57 Knockout Mice

(2015) Andrea Linz et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Intravenous Bisphosphonate Therapy of Young Children With Osteogenesis Imperfecta: Skeletal Findings During Follow Up Throughout the Growing Years

(2015) Telma Palomo et al.   JOURNAL OF BONE AND MINERAL RESEARCH

PTH Receptor Signaling in Osteoblasts Regulates Endochondral Vascularization in Maintenance of Postnatal Growth Plate

(2015) Tao Qiu et al.   JOURNAL OF BONE AND MINERAL RESEARCH

ERK1 and ERK2 Regulate Chondrocyte Terminal Differentiation During Endochondral Bone Formation

(2015) Zhijun Chen et al.   JOURNAL OF BONE AND MINERAL RESEARCH

TGFβ signaling in cartilage development and maintenance

(2014) Weiguang Wang et al.   BIRTH DEFECTS RESEARCH PART C-EMBRYO TODAY-REVIEWS

Osteogenic fate of hypertrophic chondrocytes

(2014) Guan Yang et al.   CELL RESEARCH

Mechanisms and models of endoplasmic reticulum stress in chondrodysplasia

(2014) Sara E. Patterson et al.   DEVELOPMENTAL DYNAMICS

Excessive transforming growth factor-β signaling is a common mechanism in osteogenesis imperfecta

(2014) Ingo Grafe et al.   NATURE MEDICINE

Two years’ experience with denosumab for children with Osteogenesis imperfecta type VI

(2014) Heike Hoyer-Kuhn et al.   Orphanet Journal of Rare Diseases

Hypertrophic chondrocytes can become osteoblasts and osteocytes in endochondral bone formation

(2014) L. Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SHP2 Regulates Chondrocyte Terminal Differentiation, Growth Plate Architecture and Skeletal Cell Fates

(2014) Margot E. Bowen et al.   PLoS Genetics

Chondrocytes Transdifferentiate into Osteoblasts in Endochondral Bone during Development, Postnatal Growth and Fracture Healing in Mice

(2014) Xin Zhou et al.   PLoS Genetics

Gender-Dependence of Bone Structure and Properties in Adult Osteogenesis Imperfecta Murine Model

(2013) Xiaomei Yao et al.   ANNALS OF BIOMEDICAL ENGINEERING

Deletion of beta catenin in hypertrophic growth plate chondrocytes impairs trabecular bone formation

(2013) Svitlana Golovchenko et al.   BONE

Risedronate in children with osteogenesis imperfecta: a randomised, double-blind, placebo-controlled trial

(2013) Nick Bishop et al.   LANCET

Transplanted bone marrow mononuclear cells and MSCs impart clinical benefit to children with osteogenesis imperfecta through different mechanisms

(2012) S. Otsuru et al.   BLOOD

Osterix Regulates Calcification and Degradation of Chondrogenic Matrices through Matrix Metalloproteinase 13 (MMP13) Expression in Association with Transcription Factor Runx2 during Endochondral Ossification

(2012) Riko Nishimura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Impaired Osteoblastogenesis in a Murine Model of Dominant Osteogenesis Imperfecta: A New Target for Osteogenesis Imperfecta Pharmacological Therapy

(2012) Roberta Gioia et al.   STEM CELLS

Matrix-embedded cells control osteoclast formation

(2011) Jinhu Xiong et al.   NATURE MEDICINE

New perspectives on osteogenesis imperfecta

(2011) Antonella Forlino et al.   Nature Reviews Endocrinology

Osteoblast Precursors, but Not Mature Osteoblasts, Move into Developing and Fractured Bones along with Invading Blood Vessels

(2010) Christa Maes et al.   DEVELOPMENTAL CELL

Alendronate for the Treatment of Pediatric Osteogenesis Imperfecta: A Randomized Placebo-Controlled Study

(2010) L. M. Ward et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Chaperoning osteogenesis: new protein-folding disease paradigms

(2010) Elena Makareeva et al.   TRENDS IN CELL BIOLOGY

Short-Chain HDAC Inhibitors Differentially Affect Vertebrate Development and Neuronal Chromatin

(2010) Daniel M. Fass et al.   ACS Medicinal Chemistry Letters

A randomized, controlled dose-ranging study of risedronate in children with moderate and severe osteogenesis imperfecta

(2009) Nick Bishop et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Risedronate in the Treatment of Mild Pediatric Osteogenesis Imperfecta: A Randomized Placebo-Controlled Study

(2009) Frank Rauch et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Variable bone fragility associated with an Amish COL1A2 variant and a knock-in mouse model

(2009) Ethan Daley et al.   JOURNAL OF BONE AND MINERAL RESEARCH

A robust and high-throughput Cre reporting and characterization system for the whole mouse brain

(2009) Linda Madisen et al.   NATURE NEUROSCIENCE

Targeted Induction of Endoplasmic Reticulum Stress Induces Cartilage Pathology

(2009) M. Helen Rajpar et al.   PLoS Genetics

Cellular Mechanism of Decreased Bone in Brtl Mouse Model of OI: Imbalance of Decreased Osteoblast Function and Increased Osteoclasts and Their Precursors

(2008) Thomas E Uveges et al.   JOURNAL OF BONE AND MINERAL RESEARCH