4‐PBA Treatment Improves Bone Phenotypes in the Aga2 Mouse Model of Osteogenesis Imperfecta

The Emerging Roles of Endoplasmic Reticulum Stress in Balancing Immunity and Tolerance in Health and Diseases: Mechanisms and Opportunities

(2020) Anqi Li et al.   Frontiers in Immunology

4-PBA Enhances Autophagy by Inhibiting Endoplasmic Reticulum Stress in Recombinant Human Beta Nerve Growth Factor–Induced PC12 cells After Mechanical Injury via PI3K/AKT/mTOR Signaling Pathway

(2020) Zhenyu Wang et al.   World Neurosurgery

Effect of rapamycin on bone mass and strength in the α2(I)-G610C mouse model of osteogenesis imperfecta

(2019) John F. Bateman et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Deficiency of lysyl hydroxylase 2 in mice causes systemic endoplasmic reticulum stress leading to early embryonic lethality

(2019) Atsushi Kasamatsu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

(2019) Roberta Besio et al.   Disease Models & Mechanisms

The effect of the chemical chaperone 4-phenylbutyrate on secretion and activity of the p.Q160R missense variant of coagulation factor FVII

(2019) Elisabeth Andersen et al.   Cell and Bioscience

Combination therapy in the Col1a2G610C mouse model of Osteogenesis Imperfecta reveals an additive effect of enhancing LRP5 signaling and inhibiting TGFβ signaling on trabecular bone but not on cortical bone

(2019) Shannon Kaupp et al.   BONE

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

Endoplasmic Reticulum Stress signalling - from basic mechanisms to clinical applications

(2018) Aitor Almanza et al.   FEBS Journal

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

The endoplasmic reticulum (ER) chaperone BiP is a master regulator of ER functions: Getting by with a little help from ERdj friends

(2018) Kristine Faye R. Pobre et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Effect of Anti-TGF-β Treatment in a Mouse Model of Severe Osteogenesis Imperfecta

(2018) Josephine T Tauer et al.   JOURNAL OF BONE AND MINERAL RESEARCH

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

BPS804 Anti-Sclerostin Antibody in Adults With Moderate Osteogenesis Imperfecta: Results of a Randomized Phase 2a Trial

(2017) Francis H Glorieux et al.   JOURNAL OF BONE AND MINERAL RESEARCH

4-PBA inhibits LPS-induced inflammation through regulating ER stress and autophagy in acute lung injury models

(2017) Meichun Zeng et al.   TOXICOLOGY LETTERS

Profile of sodium phenylbutyrate granules for the treatment of urea-cycle disorders: patient perspectives

(2017) Luis Peña-Quintana et al.   Patient Preference and Adherence

Transcription Factor C/EBP Homologous Protein in Health and Diseases

(2017) Yuan Yang et al.   Frontiers in Immunology

Osteogenesis imperfecta

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

Enhanced Wnt signaling improves bone mass and strength, but not brittleness, in the Col1a1 +/mov13 mouse model of type I Osteogenesis Imperfecta

(2016) Christina M. Jacobsen et al.   BONE

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

The therapeutic effects of 4-phenylbutyric acid in maintaining proteostasis

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

Molecular Consequences of theSERPINH1/HSP47 Mutation in the Dachshund Natural Model of Osteogenesis Imperfecta

(2015) Uschi Lindert et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Reduction of endoplasmic reticulum stress by 4-phenylbutyric acid prevents the development of hypoxia-induced pulmonary arterial hypertension

(2014) Masayuki Koyama et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

HSP47 and FKBP65 cooperate in the synthesis of type I procollagen

(2014) Ivan Duran et al.   HUMAN MOLECULAR GENETICS

Evaluation of teriparatide treatment in adults with osteogenesis imperfecta

(2014) Eric S. Orwoll et al.   JOURNAL OF CLINICAL INVESTIGATION

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

(2014) Ingo Grafe et al.   NATURE MEDICINE

4-Phenylbutyric acid protects against neuronal cell death by primarily acting as a chemical chaperone rather than histone deacetylase inhibitor

(2013) Seisuke Mimori et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Clinical and Experimental Applications of Sodium Phenylbutyrate

(2013) Tommaso Iannitti et al.   Drugs in research & development

Activation of the stress proteome as a mechanism for small molecule therapeutics

(2012) Rebecca Deering Brose et al.   HUMAN MOLECULAR GENETICS

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

(2012) Claudio Hetz   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Sodium Phenylbutyrate, a Drug With Known Capacity to Reduce Endoplasmic Reticulum Stress, Partially Alleviates Lipid-Induced Insulin Resistance and β-Cell Dysfunction in Humans

(2011) Changting Xiao et al.   DIABETES

Ammonia control in children with urea cycle disorders (UCDs); Phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate

(2011) Uta Lichter-Konecki et al.   MOLECULAR GENETICS AND METABOLISM

New perspectives on osteogenesis imperfecta

(2011) Antonella Forlino et al.   Nature Reviews Endocrinology

Radioprotection by the histone deacetylase inhibitor phenylbutyrate

(2011) Alexandra C. Miller et al.   RADIATION AND ENVIRONMENTAL BIOPHYSICS

Mutations in the Gene Encoding the RER Protein FKBP65 Cause Autosomal-Recessive Osteogenesis Imperfecta

(2010) Yasemin Alanay et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Homozygosity for a Missense Mutation in SERPINH1, which Encodes the Collagen Chaperone Protein HSP47, Results in Severe Recessive Osteogenesis Imperfecta

(2010) Helena E. Christiansen et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in FKBP10 cause recessive osteogenesis imperfecta and bruck syndrome

(2010) Brian P Kelley et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Bisphosphonates: Mechanism of Action and Role in Clinical Practice

(2009) Matthew T. Drake et al.   MAYO CLINIC PROCEEDINGS

Autophagic Elimination of Misfolded Procollagen Aggregates in the Endoplasmic Reticulum as a Means of Cell Protection

(2009) Yoshihito Ishida et al.   MOLECULAR BIOLOGY OF THE CELL

Phenylbutyrate Ameliorates Cognitive Deficit and Reduces Tau Pathology in an Alzheimer's Disease Mouse Model

(2009) Ana Ricobaraza et al.   NEUROPSYCHOPHARMACOLOGY

ER stress is the initial response to polyglutamine toxicity in PC12 cells

(2008) Hitoshi Nakayama et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Growth of infants with osteogenesis imperfecta treated with bisphosphonate

(2008) Kosei Hasegawa et al.   PEDIATRICS INTERNATIONAL

ER Stress-Mediated Apoptosis in a New Mouse Model of Osteogenesis imperfecta

(2008) Thomas S. Lisse et al.   PLoS Genetics