PubRank

Wolfram S Kunz

Author PubWeight™ 81.06‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Analysis of mitochondrial function in situ in permeabilized muscle fibers, tissues and cells. Nat Protoc 2008 3.47
2 Characterization of superoxide-producing sites in isolated brain mitochondria. J Biol Chem 2003 2.20
3 Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6. Neurobiol Dis 2006 1.96
4 Epilepsy, hippocampal sclerosis and febrile seizures linked by common genetic variation around SCN1A. Brain 2013 1.95
5 Recombination of human mitochondrial DNA. Science 2004 1.89
6 Parkinson phenotype in aged PINK1-deficient mice is accompanied by progressive mitochondrial dysfunction in absence of neurodegeneration. PLoS One 2009 1.71
7 Genetic variation in dopaminergic activity is associated with the risk for psychiatric side effects of levetiracetam. Epilepsia 2012 1.58
8 Genome-wide association analysis of genetic generalized epilepsies implicates susceptibility loci at 1q43, 2p16.1, 2q22.3 and 17q21.32. Hum Mol Genet 2012 1.48
9 Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies--a comment. Brain 2005 1.40
10 Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease. Nat Genet 2013 1.30
11 Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms. J Cell Biol 2008 1.25
12 Fear processing and social networking in the absence of a functional amygdala. Biol Psychiatry 2012 1.23
13 On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria. Hum Mol Genet 2003 1.13
14 Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampus. Eur J Neurosci 2002 1.12
15 Recombination of mitochondrial DNA in skeletal muscle of individuals with multiple mitochondrial DNA heteroplasmy. Nat Genet 2005 1.10
16 The mechanism of neuroprotection by topiramate in an animal model of epilepsy. Epilepsia 2004 1.09
17 Disorganization of the desmin cytoskeleton and mitochondrial dysfunction in plectin-related epidermolysis bullosa simplex with muscular dystrophy. J Neuropathol Exp Neurol 2002 1.09
18 Rare exonic deletions of the RBFOX1 gene increase risk of idiopathic generalized epilepsy. Epilepsia 2013 1.07
19 Mitochondrial potassium channels. IUBMB Life 2009 1.06
20 N-acetyl cysteine treatment rescues cognitive deficits induced by mitochondrial dysfunction in G72/G30 transgenic mice. Neuropsychopharmacology 2011 1.02
21 Primary skin fibroblasts as a model of Parkinson's disease. Mol Neurobiol 2012 1.01
22 Volumetric magnetic resonance imaging of functionally relevant structural alterations in chronic epilepsy after pilocarpine-induced status epilepticus in rats. Epilepsia 2005 1.01
23 Opening of potassium channels modulates mitochondrial function in rat skeletal muscle. Biochim Biophys Acta 2002 1.00
24 Mitochondrial involvement in temporal lobe epilepsy. Exp Neurol 2009 1.00
25 Mitochondrial DNA damage and the aging process: facts and imaginations. Free Radic Res 2006 0.99
26 Sites of generation of reactive oxygen species in homogenates of brain tissue determined with the use of respiratory substrates and inhibitors. Biochim Biophys Acta 2008 0.99
27 Exon-disrupting deletions of NRXN1 in idiopathic generalized epilepsy. Epilepsia 2013 0.99
28 Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP. Muscle Nerve 2013 0.97
29 Subfield-specific loss of hippocampal N-acetyl aspartate in temporal lobe epilepsy. Epilepsia 2007 0.96
30 Effect of coenzyme Q10 on the mitochondrial function of skin fibroblasts from Parkinson patients. J Neurol Sci 2004 0.95
31 Human epidermal keratinocytes accumulate superoxide due to low activity of Mn-SOD, leading to mitochondrial functional impairment. J Invest Dermatol 2006 0.94
32 Genome-wide linkage meta-analysis identifies susceptibility loci at 2q34 and 13q31.3 for genetic generalized epilepsies. Epilepsia 2012 0.94
33 Metabolic consequences of a novel missense mutation of the mtDNA CO I gene. Hum Mol Genet 2002 0.94
34 BK channel openers inhibit ROS production of isolated rat brain mitochondria. Exp Neurol 2008 0.92
35 Behavioral changes in G72/G30 transgenic mice. Eur Neuropsychopharmacol 2009 0.92
36 Mitochondrial dysfunction in epilepsy. Mitochondrion 2011 0.92
37 Calcium ions regulate K⁺ uptake into brain mitochondria: the evidence for a novel potassium channel. Int J Mol Sci 2009 0.91
38 Distinct patterns of mitochondrial genome diversity in bonobos (Pan paniscus) and humans. BMC Evol Biol 2010 0.91
39 Cryopreservation of mitochondria and mitochondrial function in cardiac and skeletal muscle fibers. Anal Biochem 2003 0.91
40 The contribution of thioredoxin-2 reductase and glutathione peroxidase to H(2)O(2) detoxification of rat brain mitochondria. Biochim Biophys Acta 2012 0.91
41 A novel potassium channel in skeletal muscle mitochondria. Biochim Biophys Acta 2008 0.91
42 In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T2 relaxation time and apparent diffusion coefficient. Exp Neurol 2006 0.90
43 Repeats, longevity and the sources of mtDNA deletions: evidence from 'deletional spectra'. Trends Genet 2010 0.90
44 Clonally expanded mitochondrial DNA mutations in epileptic individuals with mutated DNA polymerase gamma. J Neuropathol Exp Neurol 2008 0.90
45 Changes in mitochondrial reactive oxygen species synthesis during differentiation of skeletal muscle cells. Mitochondrion 2011 0.89
46 Mitofusin 2 mutations affect mitochondrial function by mitochondrial DNA depletion. Acta Neuropathol 2012 0.89
47 A VASP-Rac-soluble guanylyl cyclase pathway controls cGMP production in adipocytes. Sci Signal 2012 0.89
48 Microglial CD33-related Siglec-E inhibits neurotoxicity by preventing the phagocytosis-associated oxidative burst. J Neurosci 2013 0.89
49 Multidrug resistance protein-1 affects oxidative stress, endothelial dysfunction, and atherogenesis via leukotriene C4 export. Circulation 2008 0.88
50 POLG mutations cause decreased mitochondrial DNA repopulation rates following induced depletion in human fibroblasts. Biochim Biophys Acta 2010 0.87
51 Neuroimaging characteristics in mitochondrial encephalopathies associated with the m.3243A>G MTTL1 mutation. J Neurol 2012 0.87
52 Mitochondrial potassium channels and reactive oxygen species. FEBS Lett 2010 0.86
53 Binding of copper is a mechanism of homocysteine toxicity leading to COX deficiency and apoptosis in primary neurons, PC12 and SHSY-5Y cells. Neurobiol Dis 2006 0.86
54 Chapter 23 Quantification of superoxide production by mouse brain and skeletal muscle mitochondria. Methods Enzymol 2009 0.86
55 Characterization of superoxide production sites in isolated rat brain and skeletal muscle mitochondria. Biomed Pharmacother 2005 0.85
56 Mitochondrial potassium channels: from pharmacology to function. Biochim Biophys Acta 2006 0.85
57 Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis. Eur J Neurosci 2007 0.85
58 Inheritance of mitochondrial DNA recombinants in double-heteroplasmic families: potential implications for phylogenetic analysis. Am J Hum Genet 2006 0.84
59 Functional imaging of mitochondrial redox state. Methods Enzymol 2002 0.84
60 Correlation of hippocampal glucose oxidation capacity and interictal FDG-PET in temporal lobe epilepsy. Epilepsia 2003 0.84
61 Complex III-dependent superoxide production of brain mitochondria contributes to seizure-related ROS formation. Biochim Biophys Acta 2010 0.83
62 Antidiabetic sulphonylureas activate mitochondrial permeability transition in rat skeletal muscle. Br J Pharmacol 2005 0.82
63 Mitochondrial dysfunction in myofibrillar myopathy. Neuropathol Appl Neurobiol 2003 0.82
64 Apraxia of lid opening mimicking ptosis in compound heterozygosity for A467T and W748S POLG1 mutations. Mov Disord 2008 0.82
65 Mitochondrial involvement in neurodegenerative diseases. IUBMB Life 2013 0.81
66 Mutation in the mitochondrial tRNA(Ile) gene causes progressive myoclonus epilepsy. Seizure 2013 0.81
67 The cytoprotective action of the potassium channel opener BMS-191095 in C2C12 myoblasts is related to the modulation of calcium homeostasis. Cell Physiol Biochem 2010 0.81
68 Mitochondrial dysfunction in neurological disorders with epileptic phenotypes. J Bioenerg Biomembr 2010 0.81
69 Mitochondrial diseases--an expanding spectrum of disorders and affected genes. Exp Physiol 2003 0.80
70 Amelioration of water maze performance deficits by topiramate applied during pilocarpine-induced status epilepticus is negatively dose-dependent. Epilepsy Res 2006 0.79
71 Differences in flux control and reserve capacity of cytochrome c oxidase (COX) in human skeletal muscle and brain suggest different metabolic effects of mild COX deficiencies. Mol Biol Rep 2002 0.79
72 Stilbene derivatives inhibit the activity of the inner mitochondrial membrane chloride channels. Cell Mol Biol Lett 2007 0.78
73 Expression pattern of mitochondrial respiratory chain enzymes in skeletal muscle of patients harboring the A3243G point mutation or large-scale deletions of mitochondrial DNA. J Neuropathol Exp Neurol 2002 0.78
74 Concerted action of two novel tRNA mtDNA point mutations in chronic progressive external ophthalmoplegia. Biosci Rep 2008 0.77
75 Mitochondrial dysfunction due to Leber's hereditary optic neuropathy as a cause of visual loss during assessment for epilepsy surgery. Epilepsy Behav 2010 0.77
76 Hippocampal N-acetyl aspartate levels do not mirror neuronal cell densities in creatine-supplemented epileptic rats. Eur J Neurosci 2003 0.77
77 Single channel studies of the ATP-regulated potassium channel in brain mitochondria. J Bioenerg Biomembr 2009 0.77
78 Is there mitochondrial dysfunction in amyotrophic lateral sclerosis skeletal muscle? Ann Neurol 2003 0.76
79 Clonal expansion of different mtDNA variants without selective advantage in solid tumors. Mutat Res 2008 0.76
80 Diagnostic value of mitochondrial DNA mutation analysis in juvenile unilateral ptosis. Graefes Arch Clin Exp Ophthalmol 2004 0.75
81 Blood-brain barrier dysfunction can contribute to pharmacoresistance of seizures. Epilepsia 2014 0.75