Cytopathies involving mitochondrial complex II

HIFα Targeted for VHL-Mediated Destruction by Proline Hydroxylation: Implications for O ₂ Sensing

2001 • 4,537 citations

Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion

1996 • 2,667 citations

Mitochondrial reactive oxygen species trigger hypoxia-induced transcription

1998 • 1,864 citations

Reactive Oxygen Species Generated at Mitochondrial Complex III Stabilize Hypoxia-inducible Factor-1α during Hypoxia

2000 • 1,860 citations

Mutations in SDHD , a Mitochondrial Complex II Gene, in Hereditary Paraganglioma

2000 • 1,604 citations

Chemistry and Biochemistry of Flavoenzymes

2018 • 1,241 citations

Gene Mutations in the Succinate Dehydrogenase Subunit SDHB Cause Susceptibility to Familial Pheochromocytoma and to Familial Paraganglioma

2001 • 1,082 citations

Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

1993 • 1,017 citations

Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

1997 • 914 citations

Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia

1997 • 866 citations

Mitochondrial defect in Huntington's disease caudate nucleus

1996 • 737 citations

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

2001 • 714 citations

Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency

1995 • 711 citations

The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins

1999 • 686 citations

Cysteine desulfurase activity indicates a role for NIFS in metallocluster biosynthesis.

1993 • 579 citations

Ataxia with isolated vitamin E deficiency is caused by mutations in the α–tocopherol transfer protein

1995 • 561 citations

Evidence for irnnairment of energy metabofism in vivo in Huntington's disease using localized 1H NMR spectroscopy

1993 • 471 citations

Succinate: quinone oxidoreductases

1997 • 411 citations

Structure of the Escherichia coli Fumarate Reductase Respiratory Complex

1999 • 405 citations

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

2000 • 368 citations

The R22X Mutation of the SDHD Gene in Hereditary Paraganglioma Abolishes the Enzymatic Activity of Complex II in the Mitochondrial Respiratory Chain and Activates the Hypoxia Pathway

2001 • 356 citations

Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia

2000 • 333 citations

Structure of fumarate reductase from Wolinella succinogenes at 2.2 Å resolution

1999 • 332 citations

NifS-directed assembly of a transient [2Fe-2S] cluster within the NifU protein

2000 • 316 citations

Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae

1999 • 286 citations

Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in nonfamilial pheochromocytoma.

2000 • 281 citations

The Yeast Frataxin Homologue Mediates Mitochondrial Iron Efflux

1999 • 274 citations

A mitochondrial ferredoxin is essential for biogenesis of cellular iron-sulfur proteins

2000 • 269 citations

A Defect in the Cytochrome b Large Subunit in Complex II Causes Both Superoxide Anion Overproduction and Abnormal Energy Metabolism in Caenorhabditis elegans

2001 • 265 citations

Transfer of Sulfur from IscS to IscU during Fe/S Cluster Assembly

2001 • 264 citations

IscA, an Alternate Scaffold for Fe−S Cluster Biosynthesis

2001 • 241 citations

Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia

2001 • 228 citations

Dominant Isolated Renal Magnesium Loss Is Caused by Misrouting of the Na⁺,K⁺‐ATPase γ‐Subunit

2003 • 220 citations

Inhibition of Succinate Dehydrogenase by Malonic Acid Produces an “Excitotoxic” Lesion in Rat Striatum

1993 • 206 citations

Partial Inhibition of Brain Succinate Dehydrogenase by 3‐Nitropropionic Acid Is Sufficient to Initiate Striatal Degeneration in Rat

1998 • 206 citations

Biogenesis of iron–sulfur proteins in eukaryotes: a novel task of mitochondria that is inherited from bacteria

2000 • 205 citations

Iron-Sulfur Cluster Assembly

2001 • 201 citations

Yeast Mitochondrial Protein, Nfs1p, Coordinately Regulates Iron-Sulfur Cluster Proteins, Cellular Iron Uptake, and Iron Distribution

1999 • 192 citations

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

2001 • 188 citations

Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome

2000 • 171 citations

Synthetic approaches to the active sites of iron-sulfur proteins

1977 • 171 citations

Nearly all hereditary paragangliomas in The Netherlands are caused by two founder mutations in the SDHD gene

2001 • 166 citations

Combinatorial mRNA Regulation: Iron Regulatory Proteins and Iso-iron-responsive Elements (Iso-IREs)

2000 • 164 citations

Genetics and molecular biology of Huntington's disease

1995 • 160 citations

Inhibition of mitochondrial complex II induces a long-term potentiation of NMDA-mediated synaptic excitation in the striatum requiring endogenous dopamine.

2001 • 159 citations

Structural and mechanistic mapping of a unique fumarate reductase.

1999 • 153 citations

The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly

2001 • 153 citations

Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain

1999 • 147 citations

Late-onset optic atrophy, ataxia, and myopathy associated with a mutation of a complex II gene

2000 • 142 citations

Inhibition of Mitochondrial Complex II Induces a Long-Term Potentiation of NMDA-Mediated Synaptic Excitation in the Striatum Requiring Endogenous Dopamine

2001 • 137 citations

Progress in understanding structure–function relationships in respiratory chain complex II

2000 • 131 citations

A structural moDAl for the membrane‐integral domain of succinate:quinone oxidoreductases

1996 • 128 citations

Rapid suppression of free radical formation by nerve growth factor involves the mitogen-activated protein kinase pathway

1997 • 128 citations

Jac1, a mitochondrial J-type chaperone, is involved in the biogenesis of Fe/S clusters in Saccharomyces cerevisiae

2001 • 127 citations

Fumarate Reductase Mutants of Escherichia coli That Lack Covalently Bound Flavin

1989 • 110 citations

Properties of bovine heart mitochondrial cytochrome b560.

1987 • 109 citations

Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency. Abnormalities of several iron-sulfur proteins.

1993 • 90 citations

Effect of iron deficiency on succinate- and NADH-ubiquinone oxidoreductases in skeletal muscle mitochondria.

1984 • 90 citations

Structure ofL-aspartate oxidase: implications for the succinate dehydrogenase/fumarate reductase oxidoreductase family

1999 • 88 citations

Thermodynamic and electron paramagnetic resonance characterization of flavin in succinate dehydrogenase.

1981 • 88 citations

Identification of regulatory sequences in the gene for 5-aminolevulinate synthase from rat.

1993 • 85 citations

Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

2001 • 82 citations

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm

2001 • 80 citations

Kearns-Sayre syndrome and complex II deficiency

1989 • 77 citations

CCC1 Suppresses Mitochondrial Damage in the Yeast Model of Friedreich's Ataxia by Limiting Mitochondrial Iron Accumulation

2000 • 76 citations

Phenotypic dichotomy in mitochondrial complex II genetic disorders

2001 • 76 citations

Novel mutations in the SDHD gene in pedigrees with familial carotid body paraganglioma and sensorineural hearing loss

2001 • 73 citations

Effects of dietary iron deficiency on iron-sulfur proteins and bioenergetic functions of skeletal muscle mitochondria

1982 • 63 citations

Biosynthesis and membrane binding of succinate dehydrogenase in Bacillus subtilis

1980 • 63 citations

Cytochrome b in human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the components in liver mitochondria and chromosome assignment of the genes for the large (SDHC) and small (SDHD) subunits to 1q21 and 11q23

1997 • 62 citations

Promoter analysis of the human succinate dehydrogenase iron‐protein gene

1998 • 57 citations

Brain-derived neurotrophic factor inhibits apoptosis and dopamine-induced free radical production in striatal neurons but does not prevent cell death

2001 • 57 citations

Regulation of the 75-kDa Subunit of Mitochondrial Complex I by Iron

2001 • 53 citations

Transmembrane topology and axial ligands to hemes in the cytochrome b subunit of Bacillus subtilis succinate:menaquinone reductase

1995 • 47 citations

The Quinone-binding Sites of the Saccharomyces cerevisiae Succinate-ubiquinone Oxidoreductase

2001 • 43 citations

Retention of Heme in Axial Ligand Mutants of Succinate-Ubiquinone Oxidoreductase (Complex II) from Escherichia coli

2001 • 42 citations

An Escherichia coli Mutant Quinol:Fumarate Reductase Contains an EPR-detectable Semiquinone Stabilized at the Proximal Quinone-binding Site

1999 • 40 citations

The investigation of respiratory chain disorders in heart using endomyocardial biopsies

1993 • 37 citations

Multiple defects of the respiratory chain including complex II in a family with myopathy and encephalopathy

1989 • 33 citations

On the possible interelations of the reactivity of soluble succinate dehydrogenase with ferricyanide, reconstitution activity, and the hipip iron sulfur center

1975 • 30 citations

Characterization of the iron-sulfur centers in succinate dehydrogenase.

1979 • 29 citations

Single Muscle Fibre Analyses in 2 Brothers with Succinate Dehydrogenase Deficiency

1994 • 28 citations

Identification of Quinone-binding and Heme-ligating Residues of the Smallest Membrane-anchoring Subunit (QPs3) of Bovine Heart Mitochondrial Succinate:Ubiquinone Reductase

1999 • 22 citations

The gene for the iron sulfur protein of succinate dehydrogenase (SDH-IP) maps to human chromosome 1p35-36.1

1993 • 22 citations

Abortive assembly of succinate-ubiquinone reductase (Complex II) in a ferrochelatase-deficient mutant of Escherichia coli

2001 • 20 citations

The C-terminus of the succinate dehydrogenase IP peptide of Saccharomyces cerevisiae is significant for assembly of complex II

1992 • 19 citations

Succinate Dehydrogenase Deficiency

2000 • 17 citations

Resolution and Reconstitution of Succinate-Ubiquinone Reductase from Escherichia coli

1997 • 15 citations