Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5

On the Mechanism of Gating Charge Movement of ClC-5, a Human Cl−/H+ Antiporter

(2012) Giovanni Zifarelli et al.   BIOPHYSICAL JOURNAL

Proteostasis, an Emerging Therapeutic Paradigm for Managing Inflammatory Airway Stress Disease

(2012) M. Bouchecareilh et al.   CURRENT MOLECULAR MEDICINE

Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis

(2012) Melanie Thelen et al.   PLoS One

Heterogeneity in the processing of CLCN5 mutants related to Dent disease

(2011) Teddy Grand et al.   HUMAN MUTATION

ClC-5 mutations associated with Dent’s disease: a major role of the dimer interface

(2011) Stéphane Lourdel et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Potential Agents for Treating Cystic Fibrosis: Cyclic Tetrapeptides That Restore Trafficking and Activity of ΔF508-CFTR

(2011) Darren M. Hutt et al.   ACS Medicinal Chemistry Letters

Snapin Snaps into the Dynein Complex for Late Endosome-Lysosome Trafficking and Autophagy

(2010) Michisuke Yuzaki   NEURON

Structure of a Eukaryotic CLC Transporter Defines an Intermediate State in the Transport Cycle

(2010) Liang Feng et al.   SCIENCE

Novel CLCN5 mutations in patients with Dent’s disease result in altered ion currents or impaired exchanger processing

(2009) Teddy Grand et al.   KIDNEY INTERNATIONAL

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

(2009) Darren M Hutt et al.   Nature Chemical Biology