Impaired proteoglycan glycosylation, elevated TGF-β signaling, and abnormal osteoblast differentiation as the basis for bone fragility in a mouse model for gerodermia osteodysplastica

Examining tissue composition, whole-bone morphology and mechanical behavior of Gorab Prx1 mice tibiae: A mouse model of premature aging

(2017) Haisheng Yang et al.   JOURNAL OF BIOMECHANICS

TIEG1 enhances Osterix expression and mediates its induction by TGFβ and BMP2 in osteoblasts

(2016) Malayannan Subramaniam et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections

(2016) Josephina A.N. Meester et al.   GENETICS IN MEDICINE

Gorab Is Required for Dermal Condensate Cells to Respond to Hedgehog Signals during Hair Follicle Morphogenesis

(2016) Ying Liu et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Osteogenesis imperfecta

(2016) Antonella Forlino et al.   LANCET

A homozygousB3GAT3mutation causes a severe syndrome with multiple fractures, expanding the phenotype of linkeropathy syndromes

(2015) Kelly L. Jones et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

TGF-β signalopathies as a paradigm for translational medicine

(2015) Elyssa Cannaerts et al.   European Journal of Medical Genetics

N-acetylcysteine treatment ameliorates the skeletal phenotype of a mouse model of diastrophic dysplasia

(2015) Luca Monti et al.   HUMAN MOLECULAR GENETICS

Fibrillin-1 Regulates Skeletal Stem Cell Differentiation by Modulating TGFβ Activity Within the Marrow Niche

(2015) Silvia Smaldone et al.   JOURNAL OF BONE AND MINERAL RESEARCH

The golgin GMAP-210 is required for efficient membrane trafficking in the early secretory pathway

(2015) P. Roboti et al.   JOURNAL OF CELL SCIENCE

Transcriptional regulation of dentin matrix protein 1 (DMP1) in odontoblasts and osteoblasts

(2014) Alexander Jacob et al.   CONNECTIVE TISSUE RESEARCH

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

(2014) Ingo Grafe et al.   NATURE MEDICINE

Defective Initiation of Glycosaminoglycan Synthesis due to B3GALT6 Mutations Causes a Pleiotropic Ehlers-Danlos-Syndrome-like Connective Tissue Disorder

(2013) Fransiska Malfait et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The Drosophila GOLPH3 homolog regulates the biosynthesis of heparan sulfate proteoglycans by modulating the retrograde trafficking of exostosins

(2013) Wei-Ling Chang et al.   DEVELOPMENT

Effects of decorin proteoglycan on fibrillogenesis, ultrastructure, and mechanics of type I collagen gels

(2013) Shawn P. Reese et al.   MATRIX BIOLOGY

Age-dependent alterations of decorin glycosaminoglycans in human skin

(2013) Yong Li et al.   Scientific Reports

Decorin

(2012) Thomas Neill et al.   AMERICAN JOURNAL OF PATHOLOGY

Diseases of glycosylation beyond classical congenital disorders of glycosylation

(2012) Thierry Hennet   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Matrix Disruptions, Growth, and Degradation of Cartilage with Impaired Sulfation

(2012) Edward L. Mertz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

SP7 Inhibits Osteoblast Differentiation at a Late Stage in Mice

(2012) Carolina A. Yoshida et al.   PLoS One

Role of glycosaminoglycans of biglycan in BMP-2 signaling

(2011) P.A. Miguez et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Age- and gender-related alteration in plasma advanced oxidation protein products (AOPP) and glycosaminoglycan (GAG) concentrations in physiological ageing

(2011) Katarzyna Komosinska-Vassev et al.   CLINICAL CHEMISTRY AND LABORATORY MEDICINE

Regulation of RANKL-induced osteoclastogenesis by TGF-β through molecular interaction between Smad3 and Traf6

(2011) Tetsuro Yasui et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Modulation of canonical Wnt signaling by the extracellular matrix component biglycan

(2011) A. D. Berendsen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Glucocorticoids Suppress Bone Formation by Attenuating Osteoblast Differentiation via the Monomeric Glucocorticoid Receptor

(2010) Alexander Rauch et al.   Cell Metabolism

The amazing osteocyte

(2010) Lynda F Bonewald   JOURNAL OF BONE AND MINERAL RESEARCH

Proteasomal Degradation Is Transcriptionally Controlled by TCF11 via an ERAD-Dependent Feedback Loop

(2010) Janos Steffen et al.   MOLECULAR CELL

Lethal Skeletal Dysplasia in Mice and Humans Lacking the Golgin GMAP-210

(2010) Patrick Smits et al.   NEW ENGLAND JOURNAL OF MEDICINE

Dermatan sulfate domains defined by the novel antibody GD3A12, in normal tissues and ovarian adenocarcinomas

(2009) Gerdy B. ten Dam et al.   HISTOCHEMISTRY AND CELL BIOLOGY

TGF-β1–induced migration of bone mesenchymal stem cells couples bone resorption with formation

(2009) Yi Tang et al.   NATURE MEDICINE

Geroderma osteodysplasticum hereditaria and wrinkly skin syndrome in 22 patients from Oman

(2008) Anna Rajab et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Glycosaminoglycans show a specific periodic interaction with type I collagen fibrils

(2008) Mario Raspanti et al.   JOURNAL OF STRUCTURAL BIOLOGY

Decorin modulates collagen matrix assembly and mineralization

(2008) Yoshiyuki Mochida et al.   MATRIX BIOLOGY

Gerodermia osteodysplastica is caused by mutations in SCYL1BP1, a Rab-6 interacting golgin

(2008) Hans Christian Hennies et al.   NATURE GENETICS

A role for a lithium-inhibited Golgi nucleotidase in skeletal development and sulfation

(2008) J. P. Frederick et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Golgin GMAP210/TRIP11 Anchors IFT20 to the Golgi Complex

(2008) John A. Follit et al.   PLoS Genetics