Published in J Biol Chem on January 25, 1983
Mammalian mitochondrial beta-oxidation. Biochem J (1996) 2.06
Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human chromosome 1. Proc Natl Acad Sci U S A (1986) 1.84
Disorders of mitochondrial fatty acyl-CoA beta-oxidation. J Inherit Metab Dis (1999) 1.67
Isovaleric acidemia: new aspects of genetic and phenotypic heterogeneity. Am J Med Genet C Semin Med Genet (2006) 1.63
Induction of the Escherichia coli aidB gene under oxygen-limiting conditions requires a functional rpoS (katF) gene. J Bacteriol (1994) 1.50
Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients. J Clin Invest (1987) 1.49
The critical role of Arabidopsis electron-transfer flavoprotein:ubiquinone oxidoreductase during dark-induced starvation. Plant Cell (2005) 1.47
Deficiency of electron transfer flavoprotein or electron transfer flavoprotein:ubiquinone oxidoreductase in glutaric acidemia type II fibroblasts. Proc Natl Acad Sci U S A (1985) 1.45
The diverse roles of flavin coenzymes--nature's most versatile thespians. J Org Chem (2007) 1.40
Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients. J Clin Invest (1995) 1.39
Molecular heterogeneity of variant isovaleryl-CoA dehydrogenase from cultured isovaleric acidemia fibroblasts. Proc Natl Acad Sci U S A (1985) 1.34
Relationship between unusual hepatic acyl coenzyme A profiles and the pathogenesis of Reye syndrome. J Clin Invest (1988) 1.16
The mitochondrial isovaleryl-coenzyme a dehydrogenase of arabidopsis oxidizes intermediates of leucine and valine catabolism. Plant Physiol (2001) 1.15
Reactions of electron-transfer flavoprotein and electron-transfer flavoprotein: ubiquinone oxidoreductase. Biochem J (1987) 1.10
Exon skipping in IVD RNA processing in isovaleric acidemia caused by point mutations in the coding region of the IVD gene. Am J Hum Genet (2000) 1.07
Clinical variability of isovaleric acidemia in a genetically homogeneous population. J Inherit Metab Dis (2012) 1.03
Short chain acyl-coenzyme A dehydrogenase (SCAD) deficiency. Immunochemical demonstration of molecular heterogeneity due to variant SCAD with differing stability. J Clin Invest (1989) 1.02
The inborn errors of mitochondrial fatty acid oxidation. J Inherit Metab Dis (1987) 1.00
Partial purification and characterization of glutaryl-coenzyme A dehydrogenase, electron transfer flavoprotein, and electron transfer flavoprotein-Q oxidoreductase from Paracoccus denitrificans. J Bacteriol (1985) 0.98
Molecular characterization of four different classes of mutations in the isovaleryl-CoA dehydrogenase gene responsible for isovaleric acidemia. Am J Hum Genet (1991) 0.95
Nucleotide sequence of messenger RNA encoding human isovaleryl-coenzyme A dehydrogenase and its expression in isovaleric acidemia fibroblasts. J Clin Invest (1990) 0.95
Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients. J Clin Invest (1985) 0.95
Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway. J Inherit Metab Dis (2010) 0.94
Biochemical competition makes fatty-acid β-oxidation vulnerable to substrate overload. PLoS Comput Biol (2013) 0.93
Higher-plant medium- and short-chain acyl-CoA oxidases: identification, purification and characterization of two novel enzymes of eukaryotic peroxisomal beta-oxidation. Biochem J (1996) 0.92
Glutaric acidaemia type II (multiple acyl-CoA dehydrogenation deficiency). J Inherit Metab Dis (1984) 0.92
Inhibition in vitro of acyl-CoA dehydrogenases by 2-mercaptoacetate in rat liver mitochondria. Biochem J (1983) 0.92
Identification, separation, and characterization of acyl-coenzyme A dehydrogenases involved in mitochondrial beta-oxidation in higher plants Plant Physiol (1999) 0.91
Chlorpromazine and carnitine-dependency of rat liver peroxisomal beta-oxidation of long-chain fatty acids. Biochem J (1987) 0.90
Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab (2013) 0.88
Evidence for involvement of medium chain acyl-CoA dehydrogenase in the metabolism of phenylbutyrate. Mol Genet Metab (2012) 0.87
Cross-linking of the electron-transfer flavoprotein to electron-transfer flavoprotein-ubiquinone oxidoreductase with heterobifunctional reagents. Biochem J (1988) 0.86
Complementation studies of isovaleric acidemia and glutaric aciduria type II using cultured skin fibroblasts. J Clin Invest (1983) 0.86
Dynamic simulations on the mitochondrial fatty acid beta-oxidation network. BMC Syst Biol (2009) 0.83
Characterization of new ACADSB gene sequence mutations and clinical implications in patients with 2-methylbutyrylglycinuria identified by newborn screening. Mol Genet Metab (2010) 0.83
A single acyl-CoA dehydrogenase is required for catabolism of isoleucine, valine and short-chain fatty acids in Aspergillus nidulans. Fungal Genet Biol (2007) 0.83
Electron transfer flavoprotein from Methylophilus methylotrophus: properties, comparison with other electron transfer flavoproteins, and regulation of expression by carbon source. J Bacteriol (1986) 0.81
Riboflavin-responsive defects of beta-oxidation. J Inherit Metab Dis (1985) 0.80
Mitochondrial short-chain acyl-CoA dehydrogenase of human liver and kidney can function as an oxidase. Biochem J (1993) 0.79
Proteomic changes associated with deletion of the Magnaporthe oryzae conidial morphology-regulating gene COM1. Biol Direct (2010) 0.79
Induction of omega-oxidation of monocarboxylic acids in rats by acetylsalicylic acid. J Clin Invest (1991) 0.79
Fatty acyl-CoA dehydrogenase deficiency: enzyme measurement and studies on alternative metabolism. J Inherit Metab Dis (1984) 0.75
Effects of riboflavin deficiency and clofibrate treatment on the five acyl-CoA dehydrogenases in rat liver mitochondria. Biochem J (1988) 0.75
Structure and functions of the 20S and 26S proteasomes. Annu Rev Biochem (1996) 10.70
Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase. Nat Genet (2000) 7.83
T-cell-mediated regulation of osteoclastogenesis by signalling cross-talk between RANKL and IFN-gamma. Nature (2000) 6.49
SLAM (CDw150) is a cellular receptor for measles virus. Nature (2000) 6.07
A hierarchical quorum-sensing cascade in Pseudomonas aeruginosa links the transcriptional activators LasR and RhIR (VsmR) to expression of the stationary-phase sigma factor RpoS. Mol Microbiol (1996) 6.07
High-frequency transformation method and library transducing vectors for cloning mammalian cDNAs by trans-complementation of Schizosaccharomyces pombe. Nucleic Acids Res (1990) 5.00
The role of exon sequences in splice site selection. Genes Dev (1993) 4.86
A cell-specific nuclear receptor is essential for adrenal and gonadal development and sexual differentiation. Cell (1994) 4.55
Ornithine decarboxylase is degraded by the 26S proteasome without ubiquitination. Nature (1992) 4.43
Effects of nitric oxide synthase inhibitors on murine infection with Mycobacterium tuberculosis. Infect Immun (1995) 4.28
Smurf1 interacts with transforming growth factor-beta type I receptor through Smad7 and induces receptor degradation. J Biol Chem (2001) 4.22
C-Nap1, a novel centrosomal coiled-coil protein and candidate substrate of the cell cycle-regulated protein kinase Nek2. J Cell Biol (1998) 3.72
The catalytic domain of the neurofibromatosis type 1 gene product stimulates ras GTPase and complements ira mutants of S. cerevisiae. Cell (1990) 3.56
Characterization of Rose Bengal binding to sinusoidal and bile canalicular plasma membrane from rat liver. Biochim Biophys Acta (1989) 3.47
Rho1p, a yeast protein at the interface between cell polarization and morphogenesis. Science (1996) 3.42
Accelerated degradation of PML-retinoic acid receptor alpha (PML-RARA) oncoprotein by all-trans-retinoic acid in acute promyelocytic leukemia: possible role of the proteasome pathway. Cancer Res (1996) 3.37
Report on 640 victims of the Tokyo subway sarin attack. Ann Emerg Med (1996) 3.34
Germline mutation of MSH6 as the cause of hereditary nonpolyposis colorectal cancer. Nat Genet (1997) 3.34
The requirement of intestinal bacterial flora for the development of an IgE production system fully susceptible to oral tolerance induction. J Immunol (1997) 3.33
Molecular nature of colon tumors in hereditary nonpolyposis colon cancer, familial polyposis, and sporadic colon cancer. Gastroenterology (1996) 3.30
Prostaglandin D2 selectively induces chemotaxis in T helper type 2 cells, eosinophils, and basophils via seven-transmembrane receptor CRTH2. J Exp Med (2001) 3.23
The fission yeast gamma-tubulin is essential for mitosis and is localized at microtubule organizing centers. J Cell Sci (1991) 3.20
Depletion of CD4(+) T cells causes reactivation of murine persistent tuberculosis despite continued expression of interferon gamma and nitric oxide synthase 2. J Exp Med (2000) 3.20
CHIP is a chaperone-dependent E3 ligase that ubiquitylates unfolded protein. EMBO Rep (2001) 3.19
Tbx5 associates with Nkx2-5 and synergistically promotes cardiomyocyte differentiation. Nat Genet (2001) 3.14
A defect in the Kv channel-interacting protein 2 (KChIP2) gene leads to a complete loss of I(to) and confers susceptibility to ventricular tachycardia. Cell (2001) 3.13
The gene mutated in ataxia-ocular apraxia 1 encodes the new HIT/Zn-finger protein aprataxin. Nat Genet (2001) 3.01
Antigen-induced T cell-replacing factor (TRF). III. Establishment of T cell hybrid clone continuously producing TRF and functional analysis of released TRF. J Immunol (1980) 2.95
Purification and cloning of a nucleotide excision repair complex involving the xeroderma pigmentosum group C protein and a human homologue of yeast RAD23. EMBO J (1994) 2.94
NEDD8 recruits E2-ubiquitin to SCF E3 ligase. EMBO J (2001) 2.94
Columns for visual features of objects in monkey inferotemporal cortex. Nature (1992) 2.93
A class-wide phylogenetic assessment of Dothideomycetes. Stud Mycol (2009) 2.91
Neuronal selectivities to complex object features in the ventral visual pathway of the macaque cerebral cortex. J Neurophysiol (1994) 2.87
Bni1p implicated in cytoskeletal control is a putative target of Rho1p small GTP binding protein in Saccharomyces cerevisiae. EMBO J (1996) 2.87
A new NEDD8-ligating system for cullin-4A. Genes Dev (1998) 2.82
Heterothallic Behavior of a Homothallic Strain in Saccharomyces Yeast. Genetics (1958) 2.81
Bni1p and Bnr1p: downstream targets of the Rho family small G-proteins which interact with profilin and regulate actin cytoskeleton in Saccharomyces cerevisiae. EMBO J (1997) 2.80
Rom1p and Rom2p are GDP/GTP exchange proteins (GEPs) for the Rho1p small GTP binding protein in Saccharomyces cerevisiae. EMBO J (1996) 2.76
Growth site localization of Rho1 small GTP-binding protein and its involvement in bud formation in Saccharomyces cerevisiae. J Cell Biol (1994) 2.74
Targeted disruption of gp130, a common signal transducer for the interleukin 6 family of cytokines, leads to myocardial and hematological disorders. Proc Natl Acad Sci U S A (1996) 2.70
Analysis of individual human trigeminal ganglia for latent herpes simplex virus type 1 and varicella-zoster virus nucleic acids using real-time PCR. J Virol (2000) 2.70
Ataxia and epileptic seizures in mice lacking type 1 inositol 1,4,5-trisphosphate receptor. Nature (1996) 2.70
Mutanolysin, bacteriolytic agent for cariogenic Streptococci: partial purification and properties. Antimicrob Agents Chemother (1974) 2.68
Covalent modification of all members of human cullin family proteins by NEDD8. Oncogene (1999) 2.67
Portal vein anomaly associated with deviation of the ligamentum teres to the right and malposition of the gallbladder. Radiology (1998) 2.62
Impact of graft size mismatching on graft prognosis in liver transplantation from living donors. Transplantation (1999) 2.61
Characteristics of somatic mutation of the adenomatous polyposis coli gene in colorectal tumors. Cancer Res (1994) 2.61
Rapid focus reduction neutralization test of influenza A and B viruses in microtiter system. J Clin Microbiol (1990) 2.60
Biochemical and genetic studies on two different types of erythromycin resistant mutants of Escherichia coli with altered ribosomal proteins. Mol Gen Genet (1973) 2.59
Microsatellite instability in acute myelocytic leukaemia developed from A-bomb survivors. Int J Radiat Biol (2001) 2.54
Rho as a regulator of the cytoskeleton. Trends Biochem Sci (1995) 2.52
Enhanced expression of Iba1, ionized calcium-binding adapter molecule 1, after transient focal cerebral ischemia in rat brain. Stroke (2001) 2.51
Molecular analysis of the Escherichia coli hns gene encoding a DNA-binding protein, which preferentially recognizes curved DNA sequences. Mol Gen Genet (1991) 2.50
High-titer human immunodeficiency virus type 1-based vector systems for gene delivery into nondividing cells. J Virol (1998) 2.47
Expression of H-2Dd and H-2Ld mouse major histocompatibility antigen genes in L cells after DNA-mediated gene transfer. J Immunol (1983) 2.45
PAI-1 deficiency attenuates the fibrogenic response to ureteral obstruction. Kidney Int (2001) 2.41
Location of functional regions of acetylcholine receptor alpha-subunit by site-directed mutagenesis. Nature (1985) 2.40
Ampulla cardiomyopathy ('Takotusbo' cardiomyopathy)--reversible left ventricular dysfunction: with ST segment elevation. Jpn Circ J (2000) 2.39
Size and position invariance of neuronal responses in monkey inferotemporal cortex. J Neurophysiol (1995) 2.39
Human ocular dominance columns as revealed by high-field functional magnetic resonance imaging. Neuron (2001) 2.38
Structure of the 5' upstream region and the regulation of the rpoS gene of Escherichia coli. Mol Gen Genet (1994) 2.38
CDC33 encodes mRNA cap-binding protein eIF-4E of Saccharomyces cerevisiae. Mol Cell Biol (1988) 2.33
The fission yeast cut1+ gene regulates spindle pole body duplication and has homology to the budding yeast ESP1 gene. Cell (1990) 2.32
Interaction of hHR23 with S5a. The ubiquitin-like domain of hHR23 mediates interaction with S5a subunit of 26 S proteasome. J Biol Chem (1999) 2.30
Covalent modifier NEDD8 is essential for SCF ubiquitin-ligase in fission yeast. EMBO J (2000) 2.27
Isolation and characterization of a novel actin filament-binding protein from Saccharomyces cerevisiae. Oncogene (1998) 2.27
The NEDD8 system is essential for cell cycle progression and morphogenetic pathway in mice. J Cell Biol (2001) 2.26
Longitudinal study of cerebrospinal fluid levels of tau, A beta1-40, and A beta1-42(43) in Alzheimer's disease: a study in Japan. Ann Neurol (1998) 2.25
The Saccharomyces cerevisiae genes (CMP1 and CMP2) encoding calmodulin-binding proteins homologous to the catalytic subunit of mammalian protein phosphatase 2B. Mol Gen Genet (1991) 2.23
Weight loss associated with an endotoxin-induced mediator from peritoneal macrophages: the role of cachectin (tumor necrosis factor). Immunol Lett (1985) 2.23
Reversal of oncogenesis by the expression of a major histocompatibility complex class I gene. Science (1985) 2.22
Inorganic polyphosphate and the induction of rpoS expression. Proc Natl Acad Sci U S A (1997) 2.19
Weaning of immunosuppression in living donor liver transplant recipients. Transplantation (2001) 2.18
In vitro and in vivo antibacterial activities of T-3761, a new quinolone derivative. Antimicrob Agents Chemother (1993) 2.18
Intake of dairy products and calcium and prevalence of depressive symptoms during pregnancy in Japan: a cross-sectional study. BJOG (2014) 2.16
Lipopolysaccharide-treated RAW 264.7 cells produce a mediator that inhibits lipoprotein lipase in 3T3-L1 cells. J Immunol (1985) 2.15
Identity of the 19S 'prosome' particle with the large multifunctional protease complex of mammalian cells (the proteasome). Nature (1988) 2.15
Glutamate does not play a major role in controlling bone growth. J Bone Miner Res (2001) 2.12
Does stent design affect probability of restenosis? A randomized trial comparing Multilink stents with GFX stents. Am Heart J (2001) 2.11
S phase specific formation of the human Rad51 protein nuclear foci in lymphocytes. Oncogene (1996) 2.09
The centrosomal protein C-Nap1 is required for cell cycle-regulated centrosome cohesion. J Cell Biol (2000) 2.07
Selective expression of a novel surface molecule by human Th2 cells in vivo. J Immunol (1999) 2.07
Interferon-gamma induces different subunit organizations and functional diversity of proteasomes. J Biochem (1994) 2.06
Son1p is a component of the 26S proteasome of the yeast Saccharomyces cerevisiae. FEBS Lett (1998) 2.04
Insulin resistance in mitochondrial gene mutation. Diabetes Care (1994) 2.03
Analysis of local and wide-field movements in the superior temporal visual areas of the macaque monkey. J Neurosci (1986) 2.03
High frequency variation of colony morphology and chromosome reorganization in the pathogenic yeast Candida albicans. J Gen Microbiol (1989) 2.03
The SLP1 gene of Saccharomyces cerevisiae is essential for vacuolar morphogenesis and function. Mol Cell Biol (1990) 2.02
Sequence of an intestinal cDNA encoding human gastric inhibitory polypeptide precursor. Proc Natl Acad Sci U S A (1987) 2.02
Rho1p-Bni1p-Spa2p interactions: implication in localization of Bni1p at the bud site and regulation of the actin cytoskeleton in Saccharomyces cerevisiae. Mol Biol Cell (1998) 2.01
p300 gene alterations in colorectal and gastric carcinomas. Oncogene (1996) 2.01
Biosynthesis of storage proteins in developing rice seeds. Plant Physiol (1982) 2.01
Mitosis in the fission yeast Schizosaccharomyces pombe as revealed by freeze-substitution electron microscopy. J Cell Sci (1986) 2.00
VCP/p97 in abnormal protein aggregates, cytoplasmic vacuoles, and cell death, phenotypes relevant to neurodegeneration. Cell Death Differ (2001) 2.00
Isovaleric acidemia: a new genetic defect of leucine metabolism. Proc Natl Acad Sci U S A (1966) 2.00
Dwarfism and early death in mice lacking C-type natriuretic peptide. Proc Natl Acad Sci U S A (2001) 1.99
Choline kinase and phosphorylcholine phosphatase in plants. Plant Physiol (1966) 1.99