Aleksander Edelman
Responsable de l'équipe, Canalopathies épithéliales : la mucoviscidose et autres maladies
Institution:
USPC - SET - SVS
Summary
Published articles Show More
New insights into interactions between the nucleotide-binding domain of CFTR and keratin 8
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Published in Protein Science
The intermediate filament protein keratin 8 (K8) interacts with the nucleotide-binding domain 1 (NBD1) of the cystic fibrosis (CF) transmembrane regulator (CFTR) with phenylalanine 508 deletion (ΔF508), and this interaction hampers the biogenesis of functional ΔF508-CFTR and its insertion into the plasma membrane. Interruption of this interaction m...
Impact of cystinosin glycosylation on protein stability by differential dynamic SILAC
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Published in Molecular & Cellular Proteomics
Cystinosis is a rare autosomal recessive lysosomal storage disorder characterized by intralysosomal accumulation of cystine. The causative gene for cystinosis is CTNS, which encodes the protein cystinosin, a lysosomal proton-driven cystine transporter. Over 100 mutations have been reported, leading to varying disease severity, often in correlation ...
rAAV-CFTRDeltaR Rescues the Cystic Fibrosis Phenotype in Human Intestinal Organoids and Cystic Fibrosis Mice
...Published in American Journal of Respiratory and Critical Care Medicine
Rationale: Gene therapy holds promise for a curative mutation-independent treatment applicable to all patients with cystic fibrosis (CF). The various viral vector-based clinical trials conducted in the past have demonstrated safety and tolerance of different vectors, but none have led to a clear and persistent clinical benefit. Recent clinical brea...
Theses Show More
Experience
Responsable de l'équipe
université Paris Descartes
Canalopathies épithéliales : la mucoviscidose et autres maladies; Biologie cellulaire (Croissance et Signalisation); INSERM U1151 - CNRS UMR 8253, Institut Necker-Enfants Malades; Faculté de Médecine
