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

The integrated stress response regulates BMP signalling through effects on translation

(2018) Elke Malzer et al.   BMC BIOLOGY

Development of a stress response therapy targeting aggressive prostate cancer

(2018) Hao G. Nguyen et al.   Science Translational Medicine

Synergistic co-regulation and competition by a SOX9-GLI-FOXA phasic transcriptional network coordinate chondrocyte differentiation transitions

(2018) Zhijia Tan et al.   PLoS Genetics

The small molecule ISRIB rescues the stability and activity of Vanishing White Matter Disease eIF2B mutant complexes

(2018) Yao Liang Wong et al.   eLife

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

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

Rescue of impaired sociability and anxiety-like behavior in adult cacna1c-deficient mice by pharmacologically targeting eIF2α

(2017) Z D Kabir et al.   MOLECULAR PSYCHIATRY

Hydrogen sulfide protects against endoplasmic reticulum stress and mitochondrial injury in nucleus pulposus cells and ameliorates intervertebral disc degeneration

(2017) Daoliang Xu et al.   PHARMACOLOGICAL RESEARCH

Inhibition of the integrated stress response reverses cognitive deficits after traumatic brain injury

(2017) Austin Chou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Transcriptional control of chondrocyte specification and differentiation

(2017) Chia-Feng Liu et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Fibroblast growth factor 21 and its novel association with oxidative stress

(2017) Miguel Ángel Gómez-Sámano et al.   Redox Biology

The unfolded protein response in skeletal development and homeostasis

(2016) Keisuke Horiuchi et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The integrated stress response

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

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

FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders

(2016) Jenni M. Lehtonen et al.   NEUROLOGY

An Emerging Regulatory Landscape for Skeletal Development

(2016) Hironori Hojo et al.   TRENDS IN GENETICS

Cell Signaling and Stress Responses

(2016) Gökhan S. Hotamisligil et al.   Cold Spring Harbor Perspectives in Biology

Sox9-Positive Progenitor Cells Play a Key Role in Renal Tubule Epithelial Regeneration in Mice

(2016) Hyun Mi Kang et al.   Cell Reports

Nosology and classification of genetic skeletal disorders: 2015 revision

(2015) Luisa Bonafe et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Advances in Skeletal Dysplasia Genetics

(2015) Krista A. Geister et al.   Annual Review of Genomics and Human Genetics

Antioxidant and anti-inflammatory agents mitigate pathology in a mouse model of pseudoachondroplasia

(2015) Karen L. Posey et al.   HUMAN MOLECULAR GENETICS

FGF21 as a mediator of adaptive responses to stress and metabolic benefits of anti-diabetic drugs

(2015) K. H. Kim et al.   JOURNAL OF ENDOCRINOLOGY

Label-Free Quantitative Proteomics Reveals Survival Mechanisms Developed by Hypertrophic Chondrocytes under ER Stress

(2015) Mateusz Kudelko et al.   JOURNAL OF PROTEOME RESEARCH

Fibroblast growth factor (FGF21) protects mouse liver against d-galactose-induced oxidative stress and apoptosis via activating Nrf2 and PI3K/Akt pathways

(2015) Yinhang Yu et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

The transcription factors SOX9 and SOX5/SOX6 cooperate genome-wide through super-enhancers to drive chondrogenesis

(2015) Chia-Feng Liu et al.   NUCLEIC ACIDS RESEARCH

Cartilage-specific ablation of XBP1 signaling in mouse results in a chondrodysplasia characterized by reduced chondrocyte proliferation and delayed cartilage maturation and mineralization

(2015) T.L. Cameron et al.   OSTEOARTHRITIS AND CARTILAGE

Fibroblast growth factor 21 protects mouse brain against d-galactose induced aging via suppression of oxidative stress response and advanced glycation end products formation

(2015) Yinhang Yu et al.   PHARMACOLOGY BIOCHEMISTRY AND BEHAVIOR

Mutations in a translation initiation factor identify the target of a memory-enhancing compound

(2015) Y. Sekine et al.   SCIENCE

Partial restoration of protein synthesis rates by the small molecule ISRIB prevents neurodegeneration without pancreatic toxicity

(2015) M Halliday et al.   Cell Death & Disease

XBP1-Independent UPR Pathways Suppress C/EBP-β Mediated Chondrocyte Differentiation in ER-Stress Related Skeletal Disease

(2015) Trevor L. Cameron et al.   PLoS Genetics

Pharmacological dimerization and activation of the exchange factor eIF2B antagonizes the integrated stress response

(2015) Carmela Sidrauski et al.   eLife

ISMARA: automated modeling of genomic signals as a democracy of regulatory motifs

(2014) P. J. Balwierz et al.   GENOME RESEARCH

Master Regulator for Chondrogenesis, Sox9, Regulates Transcriptional Activation of the Endoplasmic Reticulum Stress Transducer BBF2H7/CREB3L2 in Chondrocytes

(2014) Kenta Hino et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Translational control of mGluR-dependent long-term depression and object-place learning by eIF2α

(2014) Gonzalo Viana Di Prisco et al.   NATURE NEUROSCIENCE

Abnormal Chondrocyte Apoptosis in the Cartilage Growth Plate is Influenced by Genetic Background and Deletion of CHOP in a Targeted Mouse Model of Pseudoachondroplasia

(2014) Katarzyna A. Piróg et al.   PLoS One

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

Opposing unfolded-protein-response signals converge on death receptor 5 to control apoptosis

(2014) M. Lu et al.   SCIENCE

Skeletal muscle mitochondrial uncoupling drives endocrine cross-talk through the induction of FGF21 as a myokine

(2013) Susanne Keipert et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Mitogen-Activated Protein Kinase Pathways in Osteoblasts

(2013) Matthew B. Greenblatt et al.   Annual Review of Cell and Developmental Biology

Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases

(2013) Claire L. Hartley et al.   HUMAN MOLECULAR GENETICS

Skeletal diseases caused by mutations that affect collagen structure and function

(2013) William V. Arnold et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Chondrocyte-Specific Pathology During Skeletal Growth and Therapeutics in a Murine Model of Pseudoachondroplasia

(2013) Karen L Posey et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Chondrocyte Proliferation Regulated by Secreted Luminal Domain of ER Stress Transducer BBF2H7/CREB3L2

(2013) Atsushi Saito et al.   MOLECULAR CELL

ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

Oral Treatment Targeting the Unfolded Protein Response Prevents Neurodegeneration and Clinical Disease in Prion-Infected Mice

(2013) J. A. Moreno et al.   Science Translational Medicine

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

Activating transcription factor 4-dependent induction of FGF21 during amino acid deprivation

(2012) Ana Luísa De Sousa-Coelho et al.   BIOCHEMICAL JOURNAL

FoxA Family Members Are Crucial Regulators of the Hypertrophic Chondrocyte Differentiation Program

(2012) Andreia Ionescu et al.   DEVELOPMENTAL CELL

Sox9 Directs Hypertrophic Maturation and Blocks Osteoblast Differentiation of Growth Plate Chondrocytes

(2012) Peter Dy et al.   DEVELOPMENTAL CELL

ER stress–mediated autophagy promotes Myc-dependent transformation and tumor growth

(2012) Lori S. Hart et al.   JOURNAL OF CLINICAL INVESTIGATION

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

Chop (Ddit3) Is Essential for D469del-COMP Retention and Cell Death in Chondrocytes in an Inducible Transgenic Mouse Model of Pseudoachondroplasia

(2011) Karen L. Posey et al.   AMERICAN JOURNAL OF PATHOLOGY

Generation of hyaline cartilaginous tissue from mouse adult dermal fibroblast culture by defined factors

(2011) Kunihiko Hiramatsu et al.   JOURNAL OF CLINICAL INVESTIGATION

Transcriptional Profiling of Chondrodysplasia Growth Plate Cartilage Reveals Adaptive ER-Stress Networks That Allow Survival but Disrupt Hypertrophy

(2011) Trevor L. Cameron et al.   PLoS One

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Understanding the role of SOX9 in acquired diseases: lessons from development

(2011) James Pritchett et al.   TRENDS IN MOLECULAR MEDICINE

SOX9 Governs Differentiation Stage-Specific Gene Expression in Growth Plate Chondrocytes via Direct Concomitant Transactivation and Repression

(2011) Victor Y. L. Leung et al.   PLoS Genetics

An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia

(2010) Seema Nundlall et al.   CELL STRESS & CHAPERONES

The binary switch that controls the life and death decisions of ER stressed β cells

(2010) Christine M Oslowski et al.   CURRENT OPINION IN CELL BIOLOGY

SOX9 is a major negative regulator of cartilage vascularization, bone marrow formation and endochondral ossification

(2010) T. Hattori et al.   DEVELOPMENT

The GCN2-ATF4 pathway is critical for tumour cell survival and proliferation in response to nutrient deprivation

(2010) Jiangbin Ye et al.   EMBO JOURNAL

In vivo cellular adaptation to ER stress: survival strategies with double-edged consequences

(2010) K. Y. Tsang et al.   JOURNAL OF CELL SCIENCE

Simple Combinations of Lineage-Determining Transcription Factors Prime cis-Regulatory Elements Required for Macrophage and B Cell Identities

(2010) Sven Heinz et al.   MOLECULAR CELL

Regulation of autophagy by ATF4 in response to severe hypoxia

(2010) T Rzymski et al.   ONCOGENE

Wolcott-Rallison syndrome

(2010) Cécile Julier et al.   Orphanet Journal of Rare Diseases

Both endoplasmic reticulum and mitochondria are involved in disc cell apoptosis and intervertebral disc degeneration in rats

(2009) Chang-Qing Zhao et al.   AGE

The unfolded protein response and its relevance to connective tissue diseases

(2009) Raymond P. Boot-Handford et al.   CELL AND TISSUE RESEARCH

Atf4 regulates chondrocyte proliferation and differentiation during endochondral ossification by activating Ihh transcription

(2009) W. Wang et al.   DEVELOPMENT

Systemic Administration of C-Type Natriuretic Peptide as a Novel Therapeutic Strategy for Skeletal Dysplasias

(2009) Akihiro Yasoda et al.   ENDOCRINOLOGY

Fibroblast Growth Factor 21 Regulates Lipolysis in White Adipose Tissue But Is Not Required for Ketogenesis and Triglyceride Clearance in Liver

(2009) Yuhei Hotta et al.   ENDOCRINOLOGY

Regulation of endoplasmic reticulum stress response by a BBF2H7-mediated Sec23a pathway is essential for chondrogenesis

(2009) Atsushi Saito et al.   NATURE CELL BIOLOGY

Data clustering: 50 years beyond K-means

(2009) Anil K. Jain   PATTERN RECOGNITION LETTERS

Ppp1r15 gene knockout reveals an essential role for translation initiation factor 2 alpha (eIF2α) dephosphorylation in mammalian development

(2009) Heather P. Harding et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeted Induction of Endoplasmic Reticulum Stress Induces Cartilage Pathology

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

Ultrafast and memory-efficient alignment of short DNA sequences to the human genome

(2009) Ben Langmead et al.   GENOME BIOLOGY

Inhibition of Growth Hormone Signaling by the Fasting-Induced Hormone FGF21

(2008) Takeshi Inagaki et al.   Cell Metabolism

Medical and social aspects of the life course for adults with a skeletal dysplasia: A review of current knowledge

(2008) Sue Thompson et al.   DISABILITY AND REHABILITATION

Matrix Remodeling During Intervertebral Disc Growth and Degeneration Detected by Multichromatic FAST Staining

(2008) Victor Y.L. Leung et al.   JOURNAL OF HISTOCHEMISTRY & CYTOCHEMISTRY

Ablation of the UPR-Mediator CHOP Restores Motor Function and Reduces Demyelination in Charcot-Marie-Tooth 1B Mice

(2008) Maria Pennuto et al.   NEURON