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

Biology of Hsp47 (Serpin H1), a collagen-specific molecular chaperone

(2017) Shinya Ito et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Functional bone histology of zebrafish reveals two types of endochondral ossification, different types of osteoblast clusters and a new bone type

(2016) Jochen Weigele et al.   JOURNAL OF ANATOMY

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

Loss of Type I Collagen Telopeptide Lysyl Hydroxylation Causes Musculoskeletal Abnormalities in a Zebrafish Model of Bruck Syndrome

(2016) Charlotte Gistelinck et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Osteogenesis imperfecta

(2016) Antonella Forlino et al.   LANCET

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

Can you teach old drugs new tricks?

(2016) Nicola Nosengo   NATURE

The spotted gar genome illuminates vertebrate evolution and facilitates human-teleost comparisons

(2016) Ingo Braasch et al.   NATURE GENETICS

Intracellular mechanisms of molecular recognition and sorting for transport of large extracellular matrix molecules

(2016) Yoshihiro Ishikawa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Zebrafish Collagen Type I: Molecular and Biochemical Characterization of the Major Structural Protein in Bone and Skin

(2016) C. Gistelinck et al.   Scientific Reports

Recent advances in the treatment of hyperammonemia

(2015) Simon Matoori et al.   ADVANCED DRUG DELIVERY REVIEWS

Mutations in SEC24D, Encoding a Component of the COPII Machinery, Cause a Syndromic Form of Osteogenesis Imperfecta

(2015) Lutz Garbes et al.   AMERICAN JOURNAL OF HUMAN GENETICS

4-PBA prevents pressure overload-induced myocardial hypertrophy and interstitial fibrosis by attenuating endoplasmic reticulum stress

(2015) Tao Luo et al.   CHEMICO-BIOLOGICAL INTERACTIONS

Morpholinos: Antisense and Sensibility

(2015) Martin Blum et al.   DEVELOPMENTAL CELL

Collagen duplicate genes of bone and cartilage participate during regeneration of zebrafish fin skeleton

(2015) I. Duran et al.   GENE EXPRESSION PATTERNS

Altered cytoskeletal organization characterized lethal but not surviving Brtl+/−mice: insight on phenotypic variability in osteogenesis imperfecta

(2015) Laura Bianchi et al.   HUMAN MOLECULAR GENETICS

Identification andIn VivoFunctional Characterization of Novel Compound HeterozygousBMP1Variants in Osteogenesis Imperfecta

(2015) Sung Yoon Cho et al.   HUMAN MUTATION

Zebrafish as tools for drug discovery

(2015) Calum A. MacRae et al.   NATURE REVIEWS DRUG DISCOVERY

New therapeutic targets in rare genetic skeletal diseases

(2015) Michael D Briggs et al.   Expert Opinion on Orphan Drugs

First Mouse Model for Combined Osteogenesis Imperfecta and Ehlers-Danlos Syndrome

(2014) Frieda Chen et al.   JOURNAL OF BONE AND MINERAL RESEARCH

The therapeutic potential of chemical chaperones in protein folding diseases

(2014) Leonardo Cortez et al.   Prion

A molecular ensemble in the rER for procollagen maturation

(2013) Yoshihiro Ishikawa et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Loss of col8a1a function during zebrafish embryogenesis results in congenital vertebral malformations

(2013) Ryan S. Gray et al.   DEVELOPMENTAL BIOLOGY

Allele-specific Col1a1 silencing reduces mutant collagen in fibroblasts from Brtl mouse, a model for classical osteogenesis imperfecta

(2013) Julie Rousseau et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Allele Dependent Silencing of Collagen Type I Using Small Interfering RNAs Targeting 3'UTR Indels - a Novel Therapeutic Approach in Osteogenesis Imperfecta

(2013) Katarina Lindahl et al.   International Journal of Medical Sciences

Generation and application of signaling pathway reporter lines in zebrafish

(2013) Enrico Moro et al.   MOLECULAR GENETICS AND GENOMICS

The zebrafish reference genome sequence and its relationship to the human genome

(2013) Kerstin Howe et al.   NATURE

Ensembl 2014

(2013) Paul Flicek et al.   NUCLEIC ACIDS RESEARCH

Clinical and Experimental Applications of Sodium Phenylbutyrate

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

Differential response to intracellular stress in the skin from osteogenesis imperfecta Brtl mice with lethal and non lethal phenotype: A proteomic approach

(2012) Laura Bianchi et al.   Journal of Proteomics

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

Actinotrichia collagens and their role in fin formation

(2011) I. Durán et al.   DEVELOPMENTAL BIOLOGY

Zebrafish Fukutin family proteins link the unfolded protein response with dystroglycanopathies

(2011) Yung-Yao Lin et al.   HUMAN MOLECULAR GENETICS

New perspectives on osteogenesis imperfecta

(2011) Antonella Forlino et al.   Nature Reviews Endocrinology

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

Autophagy eliminates a specific species of misfolded procollagen and plays a protective role in cell survival against ER stress

(2009) Yoshihito Ishida et al.   Autophagy

The unfolded protein response and its relevance to connective tissue diseases

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

Collagen IX is required for the integrity of collagen II fibrils and the regulation of vascular plexus formation in Zebrafish caudal fins

(2009) Cheng-chen Huang et al.   DEVELOPMENTAL BIOLOGY

Tracking gene expression during zebrafish osteoblast differentiation

(2009) Nan Li et al.   DEVELOPMENTAL DYNAMICS

Normal table of postembryonic zebrafish development: Staging by externally visible anatomy of the living fish

(2009) David M. Parichy et al.   DEVELOPMENTAL DYNAMICS

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

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

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

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