Impact of atypical mitochondrial cyclic-AMP level in nephropathic cystinosis

Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders

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cAMP regulates the functional activity, coupling efficiency and structural organization of mammalian F O F 1 ATP synthase

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Oxidation of NADH and ROS production by respiratory complex I

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Altered mTOR signalling in nephropathic cystinosis

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

Lysosomal cystine accumulation promotes mitochondrial depolarization and induction of redox-sensitive genes in human kidney proximal tubular cells

(2016) Rodolfo Sumayao et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

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PPARs and Mitochondrial Metabolism: From NAFLD to HCC

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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

Sirtuin 3–dependent mitochondrial dynamic improvements protect against acute kidney injury

(2015) Marina Morigi et al.   JOURNAL OF CLINICAL INVESTIGATION

Cystine accumulation attenuates insulin release from the pancreatic β-cell due to elevated oxidative stress and decreased ATP levels

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MitoLoc: A method for the simultaneous quantification of mitochondrial network morphology and membrane potential in single cells

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Cyclic AMP Mimics the Anti-ageing Effects of Calorie Restriction by Up-Regulating Sirtuin

(2015) Zhuoran Wang et al.   Scientific Reports

Slowing of neurodegeneration in Parkinson's disease and Huntington's disease: future therapeutic perspectives

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Nephropathic cystinosis: an international consensus document

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The ATP synthase: the understood, the uncertain and the unknown

(2013) John E. Walker   BIOCHEMICAL SOCIETY TRANSACTIONS

Mitochondrial Ca2+ Uptake Induces Cyclic AMP Generation in the Matrix and Modulates Organelle ATP Levels

(2013) Giulietta Di Benedetto et al.   Cell Metabolism

Cysteamine: an old drug with new potential

(2013) Martine Besouw et al.   DRUG DISCOVERY TODAY

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

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

CO2/HCO3−- and Calcium-regulated Soluble Adenylyl Cyclase as a Physiological ATP Sensor

(2013) Jonathan H. Zippin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activation of AMP kinase plays a role in the increased apoptosis in the renal proximal tubule in cystinosis

(2012) Mary Taub et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

ATP synthase oligomerization: From the enzyme models to the mitochondrial morphology

(2012) Johan Habersetzer et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Complex I deficiencies in neurological disorders

(2012) Sergio Papa et al.   TRENDS IN MOLECULAR MEDICINE

Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells

(2011) Martijn J. Wilmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Activation of the cAMP cascade in human fibroblast cultures rescues the activity of oxidatively damaged complex I

(2011) Domenico De Rasmo et al.   FREE RADICAL BIOLOGY AND MEDICINE

The SirT3 Divining Rod Points to Oxidative Stress

(2011) Eric L. Bell et al.   MOLECULAR CELL

A futile cycle, formed between two ATP-dependant γ-glutamyl cycle enzymes, γ-glutamyl cysteine synthetase and 5-oxoprolinase: the cause of cellular ATP depletion in nephrotic cystinosis?

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cGMP-Dependent Protein Kinases and cGMP Phosphodiesterases in Nitric Oxide and cGMP Action

(2010) S. H. Francis et al.   PHARMACOLOGICAL REVIEWS

Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranes

(2009) Ilka Wittig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Novel conditionally immortalized human proximal tubule cell line expressing functional influx and efflux transporters

(2009) Martijn J. Wilmer et al.   CELL AND TISSUE RESEARCH

Cyclic AMP Produced inside Mitochondria Regulates Oxidative Phosphorylation

(2009) Rebeca Acin-Perez et al.   Cell Metabolism

Mitochondrial Autophagy Promotes Cellular Injury in Nephropathic Cystinosis

(2009) P. Sansanwal et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Fixation, Mounting and Sealing with Nail Polish of Cell Specimens Lead to Incorrect FRET Measurements using Acceptor Photobleaching

(2008) Simona Rodighiero et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

Mitochondrial Complex V Expression and Activity in Cystinotic Fibroblasts

(2008) Martijn J Wilmer et al.   PEDIATRIC RESEARCH

A role for the mitochondrial deacetylase Sirt3 in regulating energy homeostasis

(2008) B.-H. Ahn et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA