Perspectives of drug-based neuroprotection targeting mitochondria

Perspectives of drug-based neuroprotection targeting mitochondria, V. Procaccio, C. Bris, J.M. Chao de la Barca, F. Oca, A. Chevrollier, P. Amati-Bonneau, D. Bonneau, P. Reynier, , Revue Neurologique, Available online 1 May 2014, ISSN 0035-3787, http://dx.doi.org/10.1016/j.neurol.2014.03.005.

Keywords: Mitochondria; Mitochondrial diseases; Neurodegenerative disorders; Pharmacological neuroprotection; Mitochondrie; Maladies mitochondriales; Maladies neurodégénératives; Neuroprotection pharmacologique; ADAlzheimer's disease; AICAR5-aminoimidazole-4-carboxamide ribonucleoside; ALSAmyotrophic lateral sclerosis; AMPAdenosine monophosphate; ATPAdenosine triphosphate; cAMPCyclic AMP; CCCPCarbonylcyanide m-chlorophenylhydrazone; CNSCentral nervous system; ERREstrogen-related receptors; ETCElectron transport chain; FAD/FADHFlavin adenine nucleotide; GDAP1Ganglioside-induced differentiation-associated protein 1; HDHuntington disease; KTPKinetin triphosphate; LHONLeber hereditary optic neuropathy; MAPKMitogen-activated protein kinases; MELASMitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes; MERRFMyoclonic epilepsy and ragged red fibers; MFN1/MFN2Mitofusin; MnSODManganese superoxide dismutase; mtDNAMitochondrial genome or DNA; mtPTPMitochondrial permeability transition pore; mTORMammalian target of rapamycin; NAD/NADHNicotinamide adenine dinucleotide; NARPNeurogenic muscle weakness, ataxia, and retinitis pigmentosa; nDNANuclear genome or DNA; NMDAAcide N-methyl-D-aspartic; NOSNitric oxide synthase; NRF1/NRF2Nuclear respiratory factors; OPA1Optic atrophy 1; OXPHOSOxidative phosphorylation; PARLPresenilins-associated rhomboid-like protein; PDParkinson's disease; PGC-1-αPPAR gamma coactivator 1-alpha; PINK1PTEN-induced putative kinase1; PKAProtein kinase A; PPARPeroxisome proliferators-activated receptors; PRCPGC1-related coactivator; CoQCoenzyme Q; RCRespiratory chain; ROSReactive oxygen species; RXRRetinoid X receptors; SIRTSirtuins; TFAMMitochondrial transcription factor A; TFB2/TFB2Transcription factors B1 and B2

Genetics of mitochondrial respiratory chain deficiencies

Genetics of mitochondrial respiratory chain deficiencies, A. Rötig, Revue Neurologique, Available online 3 May 2014, ISSN 0035-3787, http://dx.doi.org/10.1016/j.neurol.2013.11.006.

Keywords: Genetic diseases; Mitochondria; Respiratory chain; Mitochondrie; Chaîne respiratoire; Maladies génétiques

The brain is hypothermic in patients with mitochondrial diseases

The brain is hypothermic in patients with mitochondrial diseases. Mario Rango, Andrea Arighi, Cristiana Bonifati, Roberto Del Bo, Giacomo Comi and Nereo Bresolin; Journal of Cerebral Blood Flow & Metabolism (2014) 34, 915–920; doi:10.1038/jcbfm.2014.38; published online 12 March 2014

Keywords: activation; brain metabolism; brain temperature; mitochondrial diseases; recovery; visual stimulation