MitCare-2
- 4 Anni 2014/2018
- 1.000.600€ Totale Fondi
Le malattie mitocondriali sono un gruppo di malattie, spesso letali e finora incurabili, causate dal malfunzionamento dei mitocondri. Questi piccoli organuli cellulari, ereditati dalla madre, svolgono molte funzioni, inclusa quella principale di convertire il cibo ingerito con la dieta in una piccola molecola chiamata ATP che rappresenta il “combustibile” per tutte le funzioni del corpo che consumano energia. Spesso, i cambiamenti nella forma dell’organello accompagnano queste malattie e alcuni nostri promettenti risultati indicano che operando un “lifting” al mitocondrio possiamo migliorare anche l’andamento di alcuni modelli di queste malattie in laboratorio. Vogliamo dunque attaccare in più punti un circolo vizioso di cambiamenti di forma e di disfunzione mitocondriale, in modo da interrompere le conseguenze negative delle malattie mitocondriali. A questo fine selezioneremo i migliori farmaci con l’obiettivo di trasferirli al trattamento dei pazienti.
Pubblicazioni Scientifiche
- 2016-12-01 ACTA NEUROPATHOLOGICA
A neurodegenerative perspective on mitochondrial optic neuropathies
- 2015-07-01 ANNALS OF NEUROLOGY
Syndromic parkinsonism and dementia associated with OPA1 missense mutations
- 2018-03-01 CELL CALCIUM
Calcium and regulation of the mitochondrial permeability transition
- 2015-12-01 CELL DEATH & DISEASE
Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways
- 2017-12-05 CELL METABOLISM
RevAMPing Mitochondrial Shape to Live Longer
- 2016-12-13 CELL REPORTS
Optic Atrophy 1 Is Epistatic to the Core MICOS Component MIC60 in Mitochondrial Cristae Shape Control
- 2015-01-01 CURRENT MEDICINAL CHEMISTRY
Modulation of Mitochondrial Permeability Transition in Ischemia-Reperfusion Injury of the Heart. Advantages and Limitations
- 2021-01-01 DRUGS
Therapeutic Options in Hereditary Optic Neuropathies
- 2021-09-22 FRONTIERS IN NEUROLOGY
Editorial: Hereditary Optic Neuropathies: A New Perspective
- 2015-11-04 FRONTIERS IN PHYSIOLOGY
Commentary: SPG7 is an essential and conserved component of the mitochondrial permeability transition pore
- 2015-12-15 HUMAN MOLECULAR GENETICS
Targeting estrogen receptor β as preventive therapeutic strategy for Leber's hereditary optic neuropathy
- 2019-09-01 JOURNAL OF CLINICAL MEDICINE
ADCK2 Haploinsufficiency Reduces Mitochondrial Lipid Oxidation and Causes Myopathy Associated with CoQ Deficiency
- 2017-03-01 MOVEMENT DISORDERS
Mitochondrial DNA and Primary Mitochondrial Dysfunction in Parkinson's Disease
- 2019-06-12 NATURE COMMUNICATIONS
DRP1-mediated mitochondrial shape controls calcium homeostasis and muscle mass
- 2016-05-01 NATURE REVIEWS NEPHROLOGY
Mitochondrial dysfunction in inherited renal disease and acute kidney injury
- 2016-01-01 OXIDATIVE MEDICINE AND CELLULAR LONGEVITY
The Dual Function of Reactive Oxygen/Nitrogen Species in Bioenergetics and Cell Death: The Role of ATP Synthase
- 2019-07-10 OXIDATIVE MEDICINE AND CELLULAR LONGEVITY
Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells Lacking COQ6
- 2019-01-01 PHYSIOLOGICAL REVIEWS
SARCOPENIA: AGING-RELATED LOSS OF MUSCLE MASS AND FUNCTION
- 2019-04-25 SCIENTIFIC REPORTS
Vitamin K2 cannot substitute Coenzyme Q10 as electron carrier in the mitochondrial respiratory chain of mammalian cells