Published in Mitochondrion on February 22, 2012
Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion. Proc Natl Acad Sci U S A (2013) 0.96
Mitochondrial-Nuclear Epistasis Impacts Fitness and Mitochondrial Physiology of Interpopulation Caenorhabditis briggsae Hybrids. G3 (Bethesda) (2015) 0.83
Caenorhabditis elegans expressing the Saccharomyces cerevisiae NADH alternative dehydrogenase Ndi1p, as a tool to identify new genes involved in complex I related diseases. Front Genet (2015) 0.75
Paths of Heritable Mitochondrial DNA Mutation and Heteroplasmy in Reference and gas-1 Strains of Caenorhabditis elegans. Front Genet (2016) 0.75
Effectiveness of the global protected area network in representing species diversity. Nature (2004) 10.66
The status of the world's land and marine mammals: diversity, threat, and knowledge. Science (2008) 8.03
The value of the IUCN Red List for conservation. Trends Ecol Evol (2005) 4.25
Conservation. Protected areas and effective biodiversity conservation. Science (2013) 3.42
Composition and formation of intercellular junctions in epithelial cells. Science (2002) 2.86
Expanded newborn screening for inborn errors of metabolism by electrospray ionization-tandem mass spectrometry: results, outcome, and implications. Pediatrics (2003) 2.66
Pinpointing and preventing imminent extinctions. Proc Natl Acad Sci U S A (2005) 2.64
NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria. Eur J Neurosci (2002) 2.54
Protecting important sites for biodiversity contributes to meeting global conservation targets. PLoS One (2012) 2.50
Global habitat suitability models of terrestrial mammals. Philos Trans R Soc Lond B Biol Sci (2011) 2.18
Mitochondrial complex I: structure, function and pathology. J Inherit Metab Dis (2006) 2.08
Novel mutations in the NDUFS1 gene cause low residual activities in human complex I deficiencies. Mol Genet Metab (2010) 2.06
Fanconi syndrome caused by antiepileptic therapy with valproic Acid. Epilepsia (2004) 1.87
Fabry disease-often seen, rarely diagnosed. Dtsch Arztebl Int (2009) 1.85
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease. Brain (2009) 1.71
Early clinical experiences with the new influenza A (H1N1/09). Dtsch Arztebl Int (2009) 1.65
Physical exercise-induced hypoglycemia caused by failed silencing of monocarboxylate transporter 1 in pancreatic beta cells. Am J Hum Genet (2007) 1.62
The PGL family proteins associate with germ granules and function redundantly in Caenorhabditis elegans germline development. Genetics (2004) 1.61
Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease. Hum Mol Genet (2008) 1.56
ELT-2 is the predominant transcription factor controlling differentiation and function of the C. elegans intestine, from embryo to adult. Dev Biol (2008) 1.55
Inherited complex I deficiency is associated with faster protein diffusion in the matrix of moving mitochondria. Am J Physiol Cell Physiol (2008) 1.51
Pseudotumor cerebri as an important differential diagnosis of papilledema in children. Brain Dev (2005) 1.46
Rituximab as a highly effective treatment in a female adolescent with severe multiple sclerosis. Dev Med Child Neurol (2009) 1.42
The C. elegans ezrin-radixin-moesin protein ERM-1 is necessary for apical junction remodelling and tubulogenesis in the intestine. Dev Biol (2004) 1.42
Baby-walkers: an avoidable source of hazard. Lancet (2008) 1.41
Crossed cerebellar diaschisis after status epilepticus in a young child. Neuropediatrics (2012) 1.40
Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease. Am J Hum Genet (2009) 1.39
Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels. Pediatrics (2006) 1.38
Hunter disease before and during enzyme replacement therapy. Pediatr Neurol (2011) 1.38
Physical exercise-induced hyperinsulinemic hypoglycemia is an autosomal-dominant trait characterized by abnormal pyruvate-induced insulin release. Diabetes (2003) 1.33
OXPHOS mutations and neurodegeneration. EMBO J (2012) 1.33
Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry. J Pediatr (2006) 1.33
A guide to diagnosis and treatment of Leigh syndrome. J Neurol Neurosurg Psychiatry (2013) 1.31
Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity. J Biol Chem (2002) 1.27
Warfare in biodiversity hotspots. Conserv Biol (2009) 1.26
NDUFA2 complex I mutation leads to Leigh disease. Am J Hum Genet (2008) 1.18
KLP-18, a Klp2 kinesin, is required for assembly of acentrosomal meiotic spindles in Caenorhabditis elegans. Mol Biol Cell (2003) 1.16
A mitochondrial cytochrome b mutation causing severe respiratory chain enzyme deficiency in humans and yeast. FEBS J (2005) 1.15
Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol (2003) 1.12
Computer-assisted live cell analysis of mitochondrial membrane potential, morphology and calcium handling. Methods (2008) 1.11
Life cell quantification of mitochondrial membrane potential at the single organelle level. Cytometry A (2008) 1.09
beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet (2004) 1.08
Early changes in experimental osteoarthritis using the Pond-Nuki dog model: technical procedure and initial results of in vivo MR imaging. Eur Radiol (2004) 1.08
Mevalonate kinase deficiency: enlarging the clinical and biochemical spectrum. Pediatrics (2003) 1.07
Deficiency of the first mannosylation step in the N-glycosylation pathway causes congenital disorder of glycosylation type Ik. Hum Mol Genet (2004) 1.07
Public, expert and patients' opinions on preimplantation genetic diagnosis (PGD) in Germany. Reprod Biomed Online (2005) 1.07
Ribosome profiling reveals features of normal and disease-associated mitochondrial translation. Nat Commun (2013) 1.07
Improvement in left ventricular remodeling by the endothelial nitric oxide synthase enhancer AVE9488 after experimental myocardial infarction. Circulation (2008) 1.07
Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC. J Inherit Metab Dis (2011) 1.06
Complete, accurate, mammalian phylogenies aid conservation planning, but not much. Philos Trans R Soc Lond B Biol Sci (2011) 1.04
Molecular and functional analysis of apical junction formation in the gut epithelium of Caenorhabditis elegans. Dev Biol (2004) 1.04
Palivizumab-resistant human respiratory syncytial virus infection in infancy. Clin Infect Dis (2010) 1.04
Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the assembly of cytochrome c oxidase. Genome Biol (2012) 1.03
Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res (2005) 1.01
Molecular networks controlling epithelial cell polarity in development. Mech Dev (2003) 1.01
Newborn screening for hepatorenal tyrosinemia: Tandem mass spectrometric quantification of succinylacetone. Clin Chem (2006) 1.01
What is the risk of stress risers for interprosthetic fractures of the femur? A biomechanical analysis. Int Orthop (2012) 1.01
C7orf30 specifically associates with the large subunit of the mitochondrial ribosome and is involved in translation. Nucleic Acids Res (2012) 1.01
Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine. Pediatr Res (2008) 1.01
LC-MS/MS as an alternative for SDS-PAGE in blue native analysis of protein complexes. Proteomics (2009) 1.00
Increased IP3/Ca2+ signaling compensates depletion of LET-413/DLG-1 in C. elegans epithelial junction assembly. Dev Biol (2008) 0.99
Biomechanical evaluation of 3 stabilization methods on acromioclavicular joint dislocations. Am J Sports Med (2013) 0.99
Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain. J Biol Chem (2003) 0.99
A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns. PLoS One (2009) 0.99
Maintenance of the intestinal tube in Caenorhabditis elegans: the role of the intermediate filament protein IFC-2. Differentiation (2008) 0.99
Human Golgi antiapoptotic protein modulates intracellular calcium fluxes. Mol Biol Cell (2009) 0.99
Fatal glioblastoma multiforme in a patient with neurofibromatosis type I: the dilemma of systematic medical follow-up. Childs Nerv Syst (2006) 0.98
C. elegans ATAD-3 is essential for mitochondrial activity and development. PLoS One (2009) 0.98
Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I. Mol Genet Metab (2010) 0.98
Clinical, morphological, biochemical, imaging and outcome parameters in 21 individuals with mitochondrial maintenance defect related to FBXL4 mutations. J Inherit Metab Dis (2015) 0.98
Internal fixation of femoral neck fractures with posterior comminution: a biomechanical comparison of DHS® and Intertan nail®. Int Orthop (2011) 0.97
A mutation in C2orf64 causes impaired cytochrome c oxidase assembly and mitochondrial cardiomyopathy. Am J Hum Genet (2011) 0.95
Intermediate filaments in Caenorhabditis elegans. Cell Motil Cytoskeleton (2009) 0.95
Effect of the introduction of diagnosis related group systems on the distribution of admission weights in very low birthweight infants. Arch Dis Child Fetal Neonatal Ed (2010) 0.95
Waardenburg syndrome type I with heterochromia iridis and circumscribed hypopigmentation of the skin. Pediatr Dermatol (2010) 0.95
NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease. Eur J Hum Genet (2010) 0.94
Analysis of 953 human proteins from a mitochondrial HEK293 fraction by complexome profiling. PLoS One (2013) 0.94
The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency. J Mol Med (Berl) (2009) 0.93
Septo-optic dysplasia associated with a new mitochondrial cytochrome b mutation. Ann Neurol (2002) 0.93
Dyggve-Melchior-Clausen syndrome and Smith-McCort dysplasia: clinical and molecular findings in three families supporting genetic heterogeneity in Smith-McCort dysplasia. Am J Med Genet A (2006) 0.93
Lack of creatine in muscle and brain in an adult with GAMT deficiency. Ann Neurol (2003) 0.92
Predicting water sorption and volume swelling in dense polymer systems via computer simulation. J Phys Chem B (2010) 0.92
Reconciling global mammal prioritization schemes into a strategy. Philos Trans R Soc Lond B Biol Sci (2011) 0.92
Intestinal tube formation in Caenorhabditis elegans requires vang-1 and egl-15 signaling. Dev Biol (2009) 0.92
A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency. Biochim Biophys Acta (2002) 0.92
Estrogen receptor alpha expression in podocytes mediates protection against apoptosis in-vitro and in-vivo. PLoS One (2011) 0.91
Incidence and short-term outcome of children with symptomatic presentation of organic acid and fatty acid oxidation disorders in Germany. Pediatrics (2002) 0.90
In Caenorhabditis elegans nanoparticle-bio-interactions become transparent: silica-nanoparticles induce reproductive senescence. PLoS One (2009) 0.90
A patient with glycogen storage disease type Ib presenting with acute myeloid leukemia (AML) bearing monosomy 7 and translocation t(3;8)(q26;q24) after 14 years of treatment with granulocyte colony-stimulating factor (G-CSF): a case report. J Med Case Rep (2008) 0.90
Schwann cells from human neurofibromas show increased proliferation rates under the influence of progesterone. Pediatr Res (2008) 0.90
Frequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany. Eur J Pediatr (2004) 0.90
Contiguous gene deletion of ELOVL7, ERCC8 and NDUFAF2 in a patient with a fatal multisystem disorder. Hum Mol Genet (2009) 0.89
Clinical spectrum of the pseudotumor cerebri complex in children. Childs Nerv Syst (2009) 0.89
Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency. Cell Calcium (2008) 0.89
Effect of polarization on the opsin shift in rhodopsins. 1. A combined QM/QM/MM model for bacteriorhodopsin and pharaonis sensory rhodopsin II. J Phys Chem B (2008) 0.89