Mitochondrial Dynamics of Proximal Tubular Epithelial Cells in Nephropathic Cystinosis

Sirtuin 3 inhibition induces mitochondrial stress in tongue cancer by targeting mitochondrial fission and the JNK-Fis1 biological axis

(2019) Jichi Zhou et al.   CELL STRESS & CHAPERONES

Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

(2019) Sujay Guha et al.   HUMAN MOLECULAR GENETICS

The phosphorylation status of Ser-637 in dynamin-related protein 1 (Drp1) does not determine Drp1 recruitment to mitochondria

(2019) Rong Yu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Impact of atypical mitochondrial cyclic-AMP level in nephropathic cystinosis

(2018) Francesco Bellomo et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Mitochondria–lysosome contacts regulate mitochondrial fission via RAB7 GTP hydrolysis

(2018) Yvette C. Wong et al.   NATURE

Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

(2018) Beatrice Paola Festa et al.   Nature Communications

Pre-embedding labeling for subcellular detection of molecules with electron microscopy

(2018) Elena V. Polishchuk et al.   TISSUE & CELL

The short variant of the mitochondrial dynamin OPA1 maintains mitochondrial energetics and cristae structure

(2017) Hakjoo Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

High-content drug screening for rare diseases

(2017) F. Bellomo et al.   JOURNAL OF INHERITED METABOLIC DISEASE

A novel ADOA-associated OPA1 mutation alters the mitochondrial function, membrane potential, ROS production and apoptosis

(2017) Juanjuan Zhang et al.   Scientific Reports

Control of mitochondrial biogenesis and function by the ubiquitin–proteasome system

(2017) Piotr Bragoszewski et al.   Open Biology

Mito-Morphosis: Mitochondrial Fusion, Fission, and Cristae Remodeling as Key Mediators of Cellular Function

(2016) Lena Pernas et al.   Annual Review of Physiology

Altered mTOR signalling in nephropathic cystinosis

(2016) Ekaterina A. Ivanova et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Activation of the transcription factor EB rescues lysosomal abnormalities in cystinotic kidney cells

(2016) Laura R. Rega et al.   KIDNEY INTERNATIONAL

The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives

(2016) Stephanie Cherqui et al.   Nature Reviews Nephrology

Mitochondrial dysfunction in inherited renal disease and acute kidney injury

(2016) Francesco Emma et al.   Nature Reviews Nephrology

Cystinosin-LKG rescues cystine accumulation and decreases apoptosis rate in cystinotic proximal tubular epithelial cells

(2016) Anna Taranta et al.   PEDIATRIC RESEARCH

Ubiquitin-Dependent And Independent Signals In Selective Autophagy

(2016) Aliaksandr Khaminets et al.   TRENDS IN CELL BIOLOGY

Intramitochondrial adenylyl cyclase controls the turnover of nuclear-encoded subunits and activity of mammalian complex I of the respiratory chain

(2015) Domenico De Rasmo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

(2015) G. Napolitano et al.   EMBO Molecular Medicine

USP30 deubiquitylates mitochondrial Parkin substrates and restricts apoptotic cell death

(2015) J.-R. Liang et al.   EMBO REPORTS

Cystinosin is a Component of the Vacuolar H+-ATPase-Ragulator-Rag Complex Controlling Mammalian Target of Rapamycin Complex 1 Signaling

(2015) Z. Andrzejewska et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Cysteamine treatment restores the in vitro ability to differentiate along the osteoblastic lineage of mesenchymal stromal cells isolated from bone marrow of a cystinotic patient

(2015) Antonella Conforti et al.   Journal of Translational Medicine

Endo-Lysosomal Dysfunction in Human Proximal Tubular Epithelial Cells Deficient for Lysosomal Cystine Transporter Cystinosin

(2015) Ekaterina A. Ivanova et al.   PLoS One

Regulation of the biogenesis of OXPHOS complexes in cell transition from replicating to quiescent state

(2014) Anna Signorile et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Time Course of Pathogenic and Adaptation Mechanisms in Cystinotic Mouse Kidneys

(2014) H. P. Gaide Chevronnay et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Disturbed mitochondrial dynamics and neurodegenerative disorders

(2014) Florence Burté et al.   Nature Reviews Neurology

Nephropathic cystinosis: an international consensus document

(2014) F. Emma et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Dedifferentiation and aberrations of the endolysosomal compartment characterize the early stage of nephropathic cystinosis

(2013) C. Raggi et al.   HUMAN MOLECULAR GENETICS

Upregulation of the Rab27a-Dependent Trafficking and Secretory Mechanisms Improves Lysosomal Transport, Alleviates Endoplasmic Reticulum Stress, and Reduces Lysosome Overload in Cystinosis

(2013) J. L. Johnson et al.   MOLECULAR AND CELLULAR BIOLOGY

Interchangeable adaptors regulate mitochondrial dynamin assembly for membrane scission

(2013) S. Koirala et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The PINK1/Parkin pathway regulates mitochondrial dynamics and function in mammalian hippocampal and dopaminergic neurons

(2011) Wendou Yu et al.   HUMAN MOLECULAR GENETICS

cAMP-dependent protein kinase regulates post-translational processing and expression of complex I subunits in mammalian cells

(2010) Sergio Papa et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin

(2010) Atsushi Tanaka et al.   JOURNAL OF CELL BIOLOGY