ER, Mitochondria, and ISR Regulation by mt‐HSP70 and ATF5 upon Procollagen Misfolding in Osteoblasts

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

(2022) Ivan Duran et al.   JOURNAL OF BONE AND MINERAL RESEARCH

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

(2022) Amanda L. Scheiber et al.   JCI Insight

LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding

(2021) Chun-Shik Shin et al.   Nature Communications

Integrated analysis of multimodal single-cell data

(2021) Yuhan Hao et al.   CELL

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

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

SINGLE CELL RNA SEQUENCING OF CALVARIAL AND LONG BONE ENDOCORTICAL CELLS

(2020) Ugur M. Ayturk et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Mechanisms, regulation and functions of the unfolded protein response

(2020) Claudio Hetz et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mechanisms of procollagen and HSP47 sorting during ER-to-Golgi trafficking

(2020) Shakib Omari et al.   MATRIX BIOLOGY

Mitochondrial unfolded protein-related responses across kingdoms: similar problems, different regulators

(2020) Huy Cuong Tran et al.   MITOCHONDRION

Challenges in unsupervised clustering of single-cell RNA-seq data

(2019) Vladimir Yu Kiselev et al.   NATURE REVIEWS GENETICS

Posttranslational modifications and proteinopathies: how guardians of the proteome are defeated

(2019) Heidi Olzscha   BIOLOGICAL CHEMISTRY

Mendelian bone fragility disorders

(2019) Marie-Eve Robinson et al.   BONE

Osteogenesis imperfecta

(2019) Vittoria Rossi et al.   CURRENT OPINION IN PEDIATRICS

The Unmixing Problem: A Guide to Applying Single-Cell RNA Sequencing to Bone

(2019) Matthew B Greenblatt et al.   JOURNAL OF BONE AND MINERAL RESEARCH

The Hsp70 chaperone network

(2019) Rina Rosenzweig et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

CREB3 Transcription Factors: ER-Golgi Stress Transducers as Hubs for Cellular Homeostasis

(2019) Luciana Sampieri et al.   Frontiers in Cell and Developmental Biology

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

UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication

(2018) Andrew Melber et al.   CELL RESEARCH

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

Roles of the endoplasmic reticulum–resident, collagen-specific molecular chaperone Hsp47 in vertebrate cells and human disease

(2018) Shinya Ito et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Noncanonical autophagy at ER exit sites regulates procollagen turnover

(2018) Shakib Omari et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

The mitochondrial UPR: mechanisms, physiological functions and implications in ageing

(2017) Tomer Shpilka et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

ATF5 regulates β-cell survival during stress

(2017) Christine A. Juliana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Osteogenesis imperfecta

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

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

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

Makings of a brittle bone: Unexpected lessons from a low protein diet study of a mouse OI model

(2016) E.L. Mertz et al.   MATRIX BIOLOGY

The expanding roles of endoplasmic reticulum stress in virus replication and pathogenesis

(2013) Shanshan Li et al.   CRITICAL REVIEWS IN MICROBIOLOGY

Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency

(2012) Adriana Ordóñez et al.   HEPATOLOGY

Translational Homeostasis via the mRNA Cap-Binding Protein, eIF4E

(2012) Akiko Yanagiya et al.   MOLECULAR CELL

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

Evidence for Unfolded Protein Response Activation in Monocytes from Individuals with  -1 Antitrypsin Deficiency

(2010) T. P. Carroll et al.   JOURNAL OF IMMUNOLOGY

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

MAM: more than just a housekeeper

(2009) Teruo Hayashi et al.   TRENDS IN CELL BIOLOGY

ATF4-Mediated Induction of 4E-BP1 Contributes to Pancreatic β Cell Survival under Endoplasmic Reticulum Stress

(2008) Suguru Yamaguchi et al.   Cell Metabolism

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

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

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