Published in Neurobiol Dis on March 30, 2015
Powerhouse failure and oxidative damage in autosomal recessive spastic ataxia of Charlevoix-Saguenay. J Neurol (2015) 0.80
Knock-down of pantothenate kinase 2 severely affects the development of the nervous and vascular system in zebrafish, providing new insights into PKAN disease. Neurobiol Dis (2015) 0.78
Augmentation of glycolytic metabolism by meclizine is indispensable for protection of dorsal root ganglion neurons from hypoxia-induced mitochondrial compromise. Free Radic Biol Med (2016) 0.76
Coenzyme A corrects pathological defects in human neurons of PANK2-associated neurodegeneration. EMBO Mol Med (2016) 0.75
Down-regulation of coasy, the gene associated with NBIA-VI, reduces Bmp signaling, perturbs dorso-ventral patterning and alters neuronal development in zebrafish. Sci Rep (2016) 0.75
Iron overload induced death of osteoblasts in vitro: involvement of the mitochondrial apoptotic pathway. PeerJ (2016) 0.75
Metals and neurodegeneration. Neurobiol Dis (2015) 0.75
Mitochondrial respiratory chain defects in skin fibroblasts from patients with Dravet syndrome. Neurol Sci (2015) 0.75
Cisplatin Toxicity in Dorsal Root Ganglion Neurons Is Relieved by Meclizine via Diminution of Mitochondrial Compromise and Improved Clearance of DNA Damage. Mol Neurobiol (2016) 0.75
Direct Neuronal Reprogramming for Disease Modeling Studies Using Patient-Derived Neurons: What Have We Learned? Front Neurosci (2017) 0.75
Direct generation of functional dopaminergic neurons from mouse and human fibroblasts. Nature (2011) 6.21
A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome. Nat Genet (2001) 5.34
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Coenzyme A: back in action. Prog Lipid Res (2005) 2.62
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis. Cell Metab (2006) 2.21
Genotypic and phenotypic spectrum of PANK2 mutations in patients with neurodegeneration with brain iron accumulation. Ann Neurol (2006) 1.86
Redox-dependent modulation of aconitase activity in intact mitochondria. Biochemistry (2003) 1.73
Modulation of the mitochondrial permeability transition pore. Effect of protons and divalent cations. J Biol Chem (1992) 1.71
Novel histopathologic findings in molecularly-confirmed pantothenate kinase-associated neurodegeneration. Brain (2011) 1.66
Pantethine treatment is effective in recovering the disease phenotype induced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model. Brain (2013) 1.54
Deficiency of pantothenate kinase 2 (Pank2) in mice leads to retinal degeneration and azoospermia. Hum Mol Genet (2004) 1.44
Mitochondrial iron-sulfur protein biogenesis and human disease. Biochimie (2014) 1.34
Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron availability: hypothesis for a protective role in Friedreich ataxia. Hum Mol Genet (2008) 1.30
The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins. Acta Neuropathol (2007) 1.27
Hallervorden-Spatz disease: cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus. Ann Neurol (1985) 1.26
Development-inspired reprogramming of the mammalian central nervous system. Science (2014) 1.21
Iron-related MRI images in patients with pantothenate kinase-associated neurodegeneration (PKAN) treated with deferiprone: results of a phase II pilot trial. Mov Disord (2011) 1.21
fumble encodes a pantothenate kinase homolog required for proper mitosis and meiosis in Drosophila melanogaster. Genetics (2001) 1.19
Inflammation alters the expression of DMT1, FPN1 and hepcidin, and it causes iron accumulation in central nervous system cells. J Neurochem (2013) 1.15
De novo CoA biosynthesis is required to maintain DNA integrity during development of the Drosophila nervous system. Hum Mol Genet (2008) 1.14
Metabolic consequences of mitochondrial coenzyme A deficiency in patients with PANK2 mutations. Mol Genet Metab (2011) 1.13
Localization and regulation of mouse pantothenate kinase 2. FEBS Lett (2007) 1.12
A novel transferrin/TfR2-mediated mitochondrial iron transport system is disrupted in Parkinson's disease. Neurobiol Dis (2009) 1.11
Assessment of chelatable mitochondrial iron by using mitochondrion-selective fluorescent iron indicators with different iron-binding affinities. Chembiochem (2007) 1.09
Neurodegeneration with brain iron accumulation: update on pathogenic mechanisms. Front Pharmacol (2014) 1.09
Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration. Proc Natl Acad Sci U S A (2010) 1.09
Germline deletion of pantothenate kinases 1 and 2 reveals the key roles for CoA in postnatal metabolism. PLoS One (2012) 1.08
Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. Mol Cell Neurosci (2012) 1.08
Gene-environment interactions in Parkinson's disease: the importance of animal modeling. Clin Pharmacol Ther (2010) 1.07
Labile iron in cells and body fluids: physiology, pathology, and pharmacology. Front Pharmacol (2014) 1.03
Mitochondria and aging: a role for the permeability transition? Aging Cell (2004) 1.03
Impaired Coenzyme A metabolism affects histone and tubulin acetylation in Drosophila and human cell models of pantothenate kinase associated neurodegeneration. EMBO Mol Med (2011) 1.03
Mitochondrial iron metabolism and its role in neurodegeneration. J Alzheimers Dis (2010) 1.01
Iron toxicity in neurodegeneration. Biometals (2012) 1.00
The role of iron in mitochondrial function. Biochim Biophys Acta (2008) 1.00
Pantothenate kinase-associated neurodegeneration: altered mitochondria membrane potential and defective respiration in Pank2 knock-out mouse model. Hum Mol Genet (2012) 0.97
Pantothenate kinase-2 (Pank2) silencing causes cell growth reduction, cell-specific ferroportin upregulation and iron deregulation. Neurobiol Dis (2010) 0.92
Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease. Front Pharmacol (2014) 0.90
Aconitase post-translational modification as a key in linkage between Krebs cycle, iron homeostasis, redox signaling, and metabolism of reactive oxygen species. Redox Rep (2013) 0.88
Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life. J Neurol (1992) 0.87
Human L-ferritin deficiency is characterized by idiopathic generalized seizures and atypical restless leg syndrome. J Exp Med (2013) 0.86
Skin fibroblasts from pantothenate kinase-associated neurodegeneration patients show altered cellular oxidative status and have defective iron-handling properties. Hum Mol Genet (2012) 0.86
Over-expression of mitochondrial ferritin affects the JAK2/STAT5 pathway in K562 cells and causes mitochondrial iron accumulation. Haematologica (2011) 0.84
Defective pantothenate metabolism and neurodegeneration. Biochem Soc Trans (2014) 0.82
Iron-sensitive fluorescent probes: monitoring intracellular iron pools. Metallomics (2015) 0.82
The role of Fe2+-induced lipid peroxidation in the initiation of the mitochondrial permeability transition. Arch Biochem Biophys (2003) 0.82
Fe-S cluster biogenesis in isolated mammalian mitochondria: coordinated use of persulfide sulfur and iron and requirements for GTP, NADH, and ATP. J Biol Chem (2014) 0.81
Cofilin/Twinstar phosphorylation levels increase in response to impaired coenzyme a metabolism. PLoS One (2012) 0.79
Pathophysiology and treatment of neurodegeneration with brain iron accumulation in the pediatric population. Curr Treat Options Neurol (2013) 0.79
A new in vivo model of pantothenate kinase-associated neurodegeneration reveals a surprising role for transcriptional regulation in pathogenesis. Front Cell Neurosci (2013) 0.77
Induction of the mitochondrial permeability transition (MPT) by micromolar iron: liberation of calcium is more important than NAD(P)H oxidation. Biochim Biophys Acta (2012) 0.76