Published in Circulation on February 29, 2012
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster delivery. Biochim Biophys Acta (2014) 1.12
Diagnosis and management of hypertrophic cardiomyopathy. Echo Res Pract (2015) 1.07
Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation. Front Pharmacol (2014) 0.91
Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database. Brain (2013) 0.84
Longitudinal strain bull's eye plot patterns in patients with cardiomyopathy and concentric left ventricular hypertrophy. Eur J Med Res (2016) 0.78
MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis. Heart Fail Rev (2016) 0.75
Cardiomyopathy in Friedreich ataxia: exemplifying the challenges faced by cardiologists in the management of rare diseases. Circulation (2012) 0.75
Friedreich Ataxia: current status and future prospects. Cerebellum Ataxias (2017) 0.75
[Heart involvement in Friedreich's ataxia]. Herz (2014) 0.75
PML in a patient treated with fumaric acid. N Engl J Med (2013) 3.87
A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain (2011) 3.48
Impact of myocardial fibrosis in patients with symptomatic severe aortic stenosis. Circulation (2009) 2.88
Strain and strain rate imaging: a new clinical approach to quantifying regional myocardial function. J Am Soc Echocardiogr (2004) 2.84
Senataxin, the ortholog of a yeast RNA helicase, is mutant in ataxia-ocular apraxia 2. Nat Genet (2004) 2.75
Airway obstruction in systolic heart failure--COPD or congestion? Int J Cardiol (2013) 2.73
Early outcome of carotid angioplasty and stenting with and without cerebral protection devices: a systematic review of the literature. Stroke (2003) 2.63
Low-gradient aortic valve stenosis myocardial fibrosis and its influence on function and outcome. J Am Coll Cardiol (2011) 2.54
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6. EMBO J (2009) 2.37
Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse. Neuromuscul Disord (2004) 2.37
Repetitive bilateral arm training and motor cortex activation in chronic stroke: a randomized controlled trial. JAMA (2004) 2.36
Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease. Hum Mol Genet (2005) 2.28
The sequential changes in myocardial thickness and thickening which occur during acute transmural infarction, infarct reperfusion and the resultant expression of reperfusion injury. Eur Heart J (2004) 2.25
Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: analysis of baseline data. Lancet Neurol (2013) 2.24
Mammalian animal models for Duchenne muscular dystrophy. Neuromuscul Disord (2009) 2.15
Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice. Mol Cell Biol (2004) 2.15
Experimental validation of a new ultrasound method for the simultaneous assessment of radial and longitudinal myocardial deformation independent of insonation angle. Circulation (2005) 2.12
Pre-fibrillar alpha-synuclein variants with impaired beta-structure increase neurotoxicity in Parkinson's disease models. EMBO J (2009) 2.09
Long-term effects of enzyme replacement therapy on fabry cardiomyopathy: evidence for a better outcome with early treatment. Circulation (2009) 2.07
Myocardial function defined by strain rate and strain during alterations in inotropic states and heart rate. Am J Physiol Heart Circ Physiol (2002) 2.06
Incidence rates and predictors of major and minor depression in patients with heart failure. Int J Cardiol (2012) 2.05
Systematic review of early recurrent stenosis after carotid angioplasty and stenting. Stroke (2004) 2.04
Improved regional function after autologous bone marrow-derived stem cell transfer in patients with acute myocardial infarction: a randomized, double-blind strain rate imaging study. Eur Heart J (2008) 2.01
Loss of pain perception in diabetes is dependent on a receptor of the immunoglobulin superfamily. J Clin Invest (2004) 1.92
Value of fractional uric acid excretion in differential diagnosis of hyponatremic patients on diuretics. J Clin Endocrinol Metab (2008) 1.86
High-resolution structure of the rotor ring of a proton-dependent ATP synthase. Nat Struct Mol Biol (2009) 1.80
Treatment with simvastatin in normocholesterolemic patients with Alzheimer's disease: A 26-week randomized, placebo-controlled, double-blind trial. Ann Neurol (2002) 1.79
Cellular pathology of Parkinson's disease: astrocytes, microglia and inflammation. Cell Tissue Res (2004) 1.76
Short and long-term motor skill learning in an accelerated rotarod training paradigm. Neurobiol Learn Mem (2004) 1.72
Neuroprotection by hypoxic preconditioning requires sequential activation of vascular endothelial growth factor receptor and Akt. J Neurosci (2002) 1.72
Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants. FASEB J (2003) 1.70
Efficient inhibition of the Alzheimer's disease beta-secretase by membrane targeting. Science (2008) 1.67
Toward understanding response to cardiac resynchronization therapy: left ventricular dyssynchrony is only one of multiple mechanisms. Eur Heart J (2008) 1.66
Transgenic rat model of Huntington's disease. Hum Mol Genet (2003) 1.66
Catalytic and mechanical cycles in F-ATP synthases. Fourth in the Cycles Review Series. EMBO Rep (2006) 1.65
Morphologic pattern of late gadolinium enhancement in Takotsubo cardiomyopathy detected by early cardiovascular magnetic resonance. Clin Cardiol (2011) 1.65
Protection by pioglitazone in the MPTP model of Parkinson's disease correlates with I kappa B alpha induction and block of NF kappa B and iNOS activation. J Neurochem (2004) 1.61
TDP-43-mediated neuron loss in vivo requires RNA-binding activity. PLoS One (2010) 1.58
Effect of combined systolic and diastolic functional parameter assessment for differentiation of cardiac amyloidosis from other causes of concentric left ventricular hypertrophy. Circ Cardiovasc Imaging (2013) 1.54
Clinical predictors of transient ischemic attack, stroke, or death within 30 days of carotid angioplasty and stenting. Stroke (2005) 1.53
Echocardiographic evaluation of systolic and diastolic function in patients with cardiac amyloidosis. Am J Cardiol (2011) 1.53
Maternal cardiac dysfunction and remodeling in women with preeclampsia at term. Hypertension (2010) 1.53
The c15 ring of the Spirulina platensis F-ATP synthase: F1/F0 symmetry mismatch is not obligatory. EMBO Rep (2005) 1.48
Assessment of the contractile reserve in patients with intermediate coronary lesions: a strain rate imaging study validated by invasive myocardial fractional flow reserve. Eur Heart J (2007) 1.47
Control of histone H3 phosphorylation by CaMKIIδ in response to haemodynamic cardiac stress. J Pathol (2014) 1.47
Biventricular pacing in hypertrophic obstructive cardiomyopathy: a pilot study. Heart Rhythm (2010) 1.46
The different faces of echocardiographic left ventricular hypertrophy: clues to the etiology. J Am Soc Echocardiogr (2010) 1.45
Kawasaki disease presenting as cardiac tamponade with ruptured giant aneurysm of the right coronary artery. Interact Cardiovasc Thorac Surg (2009) 1.44
A new type of proton coordination in an F(1)F(o)-ATP synthase rotor ring. PLoS Biol (2010) 1.43
Assisted reproductive technologies are associated with cardiovascular remodeling in utero that persists postnatally. Circulation (2013) 1.42
Deep brain stimulation. Cell Tissue Res (2004) 1.42
Two molecular pathways initiate mitochondria-dependent dopaminergic neurodegeneration in experimental Parkinson's disease. Proc Natl Acad Sci U S A (2007) 1.41
ADP-induced platelet aggregation frequently fails to detect impaired clopidogrel-responsiveness in patients with coronary artery disease compared to a P2Y12-specific assay. Thromb Haemost (2008) 1.40
Penguins and hummingbirds: midbrain atrophy in progressive supranuclear palsy. Neurology (2006) 1.40
Microscopic rotary mechanism of ion translocation in the F(o) complex of ATP synthases. Nat Chem Biol (2010) 1.39
Cardiac resynchronization therapy can reverse abnormal myocardial strain distribution in patients with heart failure and left bundle branch block. J Am Coll Cardiol (2003) 1.38
Molecular architecture of the undecameric rotor of a bacterial Na+-ATP synthase. J Mol Biol (2002) 1.38
Complementary and incremental mortality risk prediction by cortisol and aldosterone in chronic heart failure. Circulation (2007) 1.37
Motor skill learning depends on protein synthesis in motor cortex after training. J Neurosci (2004) 1.37
Reverse epidemiology in systolic and nonsystolic heart failure: cumulative prognostic benefit of classical cardiovascular risk factors. Circ Heart Fail (2009) 1.35
Fetal growth restriction results in remodeled and less efficient hearts in children. Circulation (2010) 1.32
The variation of morphological and functional cardiac manifestation in Fabry disease: potential implications for the time course of the disease. Eur Heart J (2005) 1.32
Two-dimensional ultrasonic strain rate measurement of the human heart in vivo. IEEE Trans Ultrason Ferroelectr Freq Control (2002) 1.31
Mitochondrial protein quality control by the proteasome involves ubiquitination and the protease Omi. J Biol Chem (2008) 1.30
Myocardial dysfunction late after low-dose anthracycline treatment in asymptomatic pediatric patients. J Am Soc Echocardiogr (2007) 1.28
The multidrug resistance protein 1 (Mrp1), but not Mrp5, mediates export of glutathione and glutathione disulfide from brain astrocytes. J Neurochem (2006) 1.28
Neurodegeneration and motor dysfunction in a conditional model of Parkinson's disease. J Neurosci (2008) 1.28
Dysnatraemia in heart failure. Eur J Heart Fail (2012) 1.28
Long-term outcome of aortic dissection with patent false lumen: predictive role of entry tear size and location. Circulation (2012) 1.27
Identification of acutely ischemic myocardium using ultrasonic strain measurements. A clinical study in patients undergoing coronary angioplasty. J Am Coll Cardiol (2003) 1.26
Echocardiographic strain and strain-rate imaging: a new tool to study regional myocardial function. IEEE Trans Med Imaging (2002) 1.25
Early regional myocardial dysfunction in young patients with Duchenne muscular dystrophy. J Am Soc Echocardiogr (2008) 1.24
The c13 ring from a thermoalkaliphilic ATP synthase reveals an extended diameter due to a special structural region. J Mol Biol (2009) 1.23
Identification of CD70-mediated apoptosis of immune effector cells as a novel immune escape pathway of human glioblastoma. Cancer Res (2002) 1.23
Novel homozygous p.E64D mutation in DJ1 in early onset Parkinson disease (PARK7). Hum Mutat (2004) 1.22
Identification and functional characterization of a novel R621C mutation in the synphilin-1 gene in Parkinson's disease. Hum Mol Genet (2003) 1.22
"GOLD or lower limit of normal definition? A comparison with expert-based diagnosis of chronic obstructive pulmonary disease in a prospective cohort-study". Respir Res (2012) 1.22
A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia. Arch Neurol (2010) 1.21
Regional right ventricular dysfunction in chronic pulmonary hypertension. J Am Soc Echocardiogr (2007) 1.21
Quantification of regional left and right ventricular radial and longitudinal function in healthy children using ultrasound-based strain rate and strain imaging. J Am Soc Echocardiogr (2002) 1.20
Structure of the c(10) ring of the yeast mitochondrial ATP synthase in the open conformation. Nat Struct Mol Biol (2012) 1.20
Silencing of the Pink1 gene expression by conditional RNAi does not induce dopaminergic neuron death in mice. Int J Biol Sci (2007) 1.20
Utility and limitations of the traditional diagnostic approach to hyponatremia: a diagnostic study. Am J Med (2010) 1.18
Granulocyte-colony stimulating factor is neuroprotective in a model of Parkinson's disease. J Neurochem (2006) 1.16
Depression comorbidity in spinocerebellar ataxia. Mov Disord (2011) 1.15
Prevention of irradiation-induced glioma cell invasion by temozolomide involves caspase 3 activity and cleavage of focal adhesion kinase. Cancer Res (2002) 1.15
Lesion location alters brain activation in chronically impaired stroke survivors. Neuroimage (2004) 1.14
A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy. Hum Mol Genet (2002) 1.14
Relation between regional functional MRI activation and vascular reactivity to carbon dioxide during normal aging. J Cereb Blood Flow Metab (2003) 1.14
Diagnostic hallmarks and pitfalls in late-onset progressive transthyretin-related amyloid-neuropathy. J Neurol (2013) 1.14
Copeptin in the differential diagnosis of hyponatremia. J Clin Endocrinol Metab (2008) 1.13
Feasibility of prehospital teleconsultation in acute stroke--a pilot study in clinical routine. PLoS One (2012) 1.12
Acute cardiac functional and morphological changes after Anthracycline infusions in children. Am J Cardiol (2007) 1.12
Rationale and design of a randomised, controlled, multicenter trial investigating the effects of selective serotonin re-uptake inhibition on morbidity, mortality and mood in depressed heart failure patients (MOOD-HF). Eur J Heart Fail (2007) 1.12
Progression of left ventricular functional abnormalities in hypertensive patients with heart failure: an ultrasonic two-dimensional speckle tracking study. J Am Soc Echocardiogr (2008) 1.11