A5041986818- Hearing, Cochlea, Tinnitus, Genetics
- Proteoglycans and glycosaminoglycans research
- Ubiquitin and proteasome pathways
- Fibroblast Growth Factor Research
- Cancer-related molecular mechanisms research
- Marine animal studies overview
- Autophagy in Disease and Therapy
- Connective tissue disorders research
- Animal Vocal Communication and Behavior
- Histone Deacetylase Inhibitors Research
- Animal Genetics and Reproduction
- Congenital heart defects research
- Protein Tyrosine Phosphatases
- Cancer-related Molecular Pathways
- Neonatal Respiratory Health Research
- Congenital Ear and Nasal Anomalies
- Peptidase Inhibition and Analysis
- ATP Synthase and ATPases Research
- Plant Molecular Biology Research
- Wnt/β-catenin signaling in development and cancer
- Cancer therapeutics and mechanisms
- Glioma Diagnosis and Treatment
- Genetics and Neurodevelopmental Disorders
- Medical Research and Treatments
- RNA Research and Splicing
University of Liège
2016-2021
Zero to Three
2014
University College London
2009-2012
University of York
2004-2008
University of Pennsylvania
2004
The signaling activities of multiple developmental ligands require sulfated heparan sulfate (HS) proteoglycans as coreceptors. QSulf1 and its mammalian orthologs are cell surface HS 6- O -endosulfatases that expressed in embryonic mesodermal neural progenitors promote Wnt signal transduction. In this study, we have investigated the function fibroblast growth factor (FGF) signaling, which requires 6-O-sulfated for FGF receptor (FGFR) dimerization tyrosine kinase activation. Here, report...
The axonal transport of vesicles promotes microtubule acetylation across species.
During embryonic development, hair cells and support in the sensory epithelia of inner ear derive from progenitors that express Sox2, a member SoxB1 family transcription factors. Sox2 is essential for specification, but high levels expression appear to inhibit cell differentiation, suggesting factors regulating activity could be critical both processes. Antagonistic interactions between SoxB2 are known regulate differentiation neural tissue, which led us investigate potential roles Sox21...
Inner ear morphogenesis is tightly regulated by the temporally and spatially coordinated action of signaling ligands their receptors. Ligand-receptor interactions are influenced heparan sulfate proteoglycans (HSPGs), cell surface molecules that consist glycosaminoglycan chains bound to a protein core. Diversity in sulfation pattern within creates binding sites for numerous factors, whose activities distribution modified association with HSPGs.Here we describe expression patterns two...
The vertebrate basic helix-loop-helix transcription factor Atoh1 is essential for maturation and survival of mechanosensory hair cells the inner ear, neurogenesis, differentiation intestine, homeostasis colon implicated in cancer progression. Given that mutations are detected malignant tumours, study functionally different alleles homologues might yield useful avenues investigation. predicted sequence chicken (cAtoh1) has large regions dissimilarity to mammalian homologues. We hypothesise...
Glioblastoma (GBM) is the most frequent and aggressive primary tumor in central nervous system. Previously, secretion of CXCL12 brain subventricular zones has been shown to attract GBM cells protect against irradiation. However, exact molecular mechanism behind this radioprotection still unknown. Here, we demonstrate that modulates phosphorylation MAP kinases their regulator, nuclear kinase phosphatase 1 (MKP1). We further show MKP1 able decrease cell death promote DNA repair after...
Mental deficiency, epilepsy, hypogonadism, microcephaly, and obesity syndrome is a severe X-linked caused by pathogenic variants in EIF2S3. The gene encodes the γ subunit of eukaryotic translation initiation factor-2, eIF2, essential for protein translation. A recurrent frameshift variant described severely affected patients while missense usually cause moderate phenotype. We identified novel (c.433A>G, p.(Met145Val)) EIF2S3 mildly patient. Studies on zebrafish confirm pathogenicity this...
Microtubules are polymerized dimers of α- and β-tubulin that underlie a broad range cellular activities. Acetylation α-tubulin by the acetyl-transferase ATAT1 modulates microtubule dynamics functions in neurons. However, it remains unclear how why this enzyme acetylates microtubules over long distances axons. Here, we show loss impairs axonal transport neurons cell free motility assays confirm requirement tubulin acetylation for proper bidirectional vesicular transport. Moreover, demonstrate...
Event Abstract Back to Regulation of proteostasis by Elp3 is crucial for sensory cell polarity in the developing inner ear. Stephen D. Freeman1*, Susana Mateo Sanchez1, Ronald L. Pouyo1, Laurence Delacroix1 and Brigitte Malgrange1 1 University Liège, Belgium The polarised alignment cells across a tissue plane, namely planar (PCP), essential integration into tissues. In ear, organ Corti exhibits exquisite polarity, most obviously observed V-shaped stereocilia hair bundles all pointing same,...
Event Abstract Back to Gene expression pattern of NEDD4-1/2 in the developing inner ear. Ronald L. Pouyo1*, Amandine Czajkowski1, Laurence Delacroix1, Stephen Freeman1 and Brigitte Malgrange1 1 Neurodevelopmental biology – GIGA Neurosciences. University Liege, Belgium Protein ubiquitination is an important post-translational modification that involved multiple cellular processes such as protein degradation, endocytosis, sorting, trafficking turnover. A recently identified mutation human...