PubRank

Iain P Hargreaves

Author PubWeight™ 27.15‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Statins and myopathy. Lancet 2002 2.21
2 A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease. Am J Hum Genet 2009 1.71
3 Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's disease. Cell Metab 2013 1.32
4 Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard. Clin Chem 2005 1.16
5 Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres. Hum Mol Genet 2010 1.10
6 ANO10 mutations cause ataxia and coenzyme Q₁₀ deficiency. J Neurol 2014 1.07
7 HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase. Orphanet J Rare Dis 2013 1.03
8 The ketogenic diet component decanoic acid increases mitochondrial citrate synthase and complex I activity in neuronal cells. J Neurochem 2014 1.01
9 Altered skeletal muscle insulin signaling and mitochondrial complex II-III linked activity in adult offspring of obese mice. Am J Physiol Regul Integr Comp Physiol 2009 0.98
10 A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy. Neuromuscul Disord 2009 0.97
11 Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment. J Inherit Metab Dis 2012 0.94
12 Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells. Pediatr Nephrol 2006 0.92
13 Mitochondrial dysfunction associated with neuronal death following status epilepticus in rat. Epilepsy Res 2002 0.89
14 Coenzyme Q10 prevents accelerated cardiac aging in a rat model of poor maternal nutrition and accelerated postnatal growth. Mol Metab 2013 0.88
15 Phenotypic variability of mitochondrial disease caused by a nuclear mutation in complex II. Mol Genet Metab 2006 0.88
16 Autosomal-recessive cerebellar ataxia caused by a novel ADCK3 mutation that elongates the protein: clinical, genetic and biochemical characterisation. J Neurol Neurosurg Psychiatry 2013 0.88
17 Neuropathologic Characterization of Pontocerebellar Hypoplasia Type 6 Associated With Cardiomyopathy and Hydrops Fetalis and Severe Multisystem Respiratory Chain Deficiency due to Novel RARS2 Mutations. J Neuropathol Exp Neurol 2015 0.85
18 Decreased ubiquinone availability and impaired mitochondrial cytochrome oxidase activity associated with statin treatment. Toxicol Mech Methods 2009 0.83
19 Coenzyme Q₁₀ deficiency in mitochondrial DNA depletion syndromes. Mitochondrion 2013 0.83
20 Dopamine but not l-dopa stimulates neural glutathione metabolism. Potential implications for Parkinson's and other dopamine deficiency states. Neurochem Int 2012 0.82
21 The pathogenic m.3243A>T mitochondrial DNA mutation is associated with a variable neurological phenotype. Neuromuscul Disord 2010 0.81
22 Absence of intracellular ion channels TPC1 and TPC2 leads to mature-onset obesity in male mice, due to impaired lipid availability for thermogenesis in brown adipose tissue. Endocrinology 2014 0.81
23 Zellweger syndrome and secondary mitochondrial myopathy. Eur J Pediatr 2014 0.81
24 Some observations upon biochemical causes of ataxia and a new disease entity ubiquinone, CoQ10 deficiency. Neurochem Res 2006 0.80
25 Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy. J Med Genet 2011 0.80
26 Coenzyme Q10 quantification in muscle, fibroblasts and cerebrospinal fluid by liquid chromatography/tandem mass spectrometry using a novel deuterated internal standard. Rapid Commun Mass Spectrom 2013 0.80
27 A recessive homozygous p.Asp92Gly SDHD mutation causes prenatal cardiomyopathy and a severe mitochondrial complex II deficiency. Hum Genet 2015 0.79
28 Allogeneic stem cell transplantation corrects biochemical derangements in MNGIE. Neurology 2007 0.75
29 Protection of respiratory chain enzymes from ischaemic damage in adult rat brain slices. Neurochem Res 2008 0.75
30 Bezafibrate induced increase in mitochondrial electron transport chain complex IV activity in human astrocytoma cells: Implications for mitochondrial cytopathies and neurodegenerative diseases. Biofactors 2010 0.75
31 Levels of 5-methyltetrahydrofolate and ascorbic acid in cerebrospinal fluid are correlated: implications for the accelerated degradation of folate by reactive oxygen species. Neurochem Int 2013 0.75