Published in Biochem Biophys Res Commun on September 02, 2005
Structure and function of biotin-dependent carboxylases. Cell Mol Life Sci (2012) 1.46
Translocation of proteins into mitochondria. Annu Rev Biochem (2007) 7.53
A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell (2005) 4.41
Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria. N Engl J Med (2002) 3.98
Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease. Nat Med (2007) 2.63
Disulfide formation in the ER and mitochondria: two solutions to a common process. Science (2009) 1.97
Ribosome binding to the Oxa1 complex facilitates co-translational protein insertion in mitochondria. EMBO J (2003) 1.79
Systematic analysis of the twin cx(9)c protein family. J Mol Biol (2009) 1.75
'Classical' organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: long-term outcome and effects of expanded newborn screening using tandem mass spectrometry. J Inherit Metab Dis (2006) 1.69
Population spectrum of ACADM genotypes correlated to biochemical phenotypes in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency. Hum Mutat (2005) 1.68
The disulfide relay system of mitochondria is connected to the respiratory chain. J Cell Biol (2007) 1.66
Pontocerebellar hypoplasia type 1: clinical spectrum and relevance of EXOSC3 mutations. Neurology (2013) 1.61
Early molecular response predicts outcomes in patients with chronic myeloid leukemia in chronic phase treated with frontline nilotinib or imatinib. Blood (2013) 1.60
Mia40, a novel factor for protein import into the intermembrane space of mitochondria is able to bind metal ions. FEBS Lett (2005) 1.59
Mba1, a membrane-associated ribosome receptor in mitochondria. EMBO J (2006) 1.50
Significant genetic differentiation between Poland and Germany follows present-day political borders, as revealed by Y-chromosome analysis. Hum Genet (2005) 1.47
Knowledge Discovery and interactive Data Mining in Bioinformatics--State-of-the-Art, future challenges and research directions. BMC Bioinformatics (2014) 1.41
Light, temperature, and desiccation effects on photosynthetic activity, and drought-induced ultrastructural changes in the green alga Klebsormidium dissectum (Streptophyta) from a high alpine soil crust. Microb Ecol (2011) 1.40
Effects of duration and amount of lung stretch at biophysical, biochemical, histological, and transcriptional levels in an in vivo rabbit model of mild lung injury. Am J Perinatol (2007) 1.39
Mitochondrial disulfide bond formation is driven by intersubunit electron transfer in Erv1 and proofread by glutathione. Mol Cell (2010) 1.38
Symptoms in carriers of adrenoleukodystrophy relate to skewed X inactivation. Ann Neurol (2002) 1.37
Plasmolysis effects and osmotic potential of two phylogenetically distinct alpine strains of Klebsormidium (Streptophyta). Protoplasma (2011) 1.34
Fluorescent chlorophyll catabolites in bananas light up blue halos of cell death. Proc Natl Acad Sci U S A (2009) 1.32
Disease manifestations and X inactivation in heterozygous females with Fabry disease. Acta Paediatr Suppl (2006) 1.30
Evolution of mitochondrial oxa proteins from bacterial YidC. Inherited and acquired functions of a conserved protein insertion machinery. J Biol Chem (2005) 1.28
Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells. Respir Res (2011) 1.28
The Oxa1 protein forms a homooligomeric complex and is an essential part of the mitochondrial export translocase in Neurospora crassa. J Biol Chem (2002) 1.21
Co-translational membrane insertion of mitochondrially encoded proteins. Biochim Biophys Acta (2009) 1.20
Osmotic stress in Arctic and Antarctic strains of the green alga Zygnema (Zygnematales, Streptophyta): effects on photosynthesis and ultrastructure. Micron (2012) 1.18
Tim17p regulates the twin pore structure and voltage gating of the mitochondrial protein import complex TIM23. J Biol Chem (2006) 1.15
Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. J Biol Chem (2004) 1.13
Cbp3-Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochrome b synthesis and assembly. J Cell Biol (2011) 1.12
Suppression of coatomer mutants by a new protein family with COPI and COPII binding motifs in Saccharomyces cerevisiae. Mol Biol Cell (2003) 1.12
Import of small Tim proteins into the mitochondrial intermembrane space. EMBO J (2003) 1.12
Changes in phenolic compounds and cellular ultrastructure of arctic and antarctic strains of Zygnema (Zygnematophyceae, Streptophyta) after exposure to experimentally enhanced UV to PAR ratio. Microb Ecol (2012) 1.12
Proteins at the polypeptide tunnel exit of the yeast mitochondrial ribosome. J Biol Chem (2010) 1.11
A novel blue fluorescent chlorophyll catabolite accumulates in senescent leaves of the peace lily and indicates a split path of chlorophyll breakdown. FEBS Lett (2010) 1.08
Mrpl36 is important for generation of assembly competent proteins during mitochondrial translation. Mol Biol Cell (2009) 1.08
The sulfhydryl oxidase Erv1 is a substrate of the Mia40-dependent protein translocation pathway. FEBS Lett (2007) 1.07
Dehydration, temperature, and light tolerance in members of the aeroterrestrial green algal genus interfilum (streptophyta) from biogeographically different temperate soils. J Phycol (2014) 1.07
Wave reflection quantification based on pressure waveforms alone--methods, comparison, and clinical covariates. Comput Methods Programs Biomed (2012) 1.06
The zinc-binding protein Hot13 promotes oxidation of the mitochondrial import receptor Mia40. EMBO Rep (2008) 1.06
Independent gene duplications of the YidC/Oxa/Alb3 family enabled a specialized cotranslational function. Proc Natl Acad Sci U S A (2009) 1.04
Advances in analytical mass spectrometry to improve screening for inherited metabolic diseases. Eur J Pediatr (2003) 1.04
Newborn screening for isovaleric acidemia using tandem mass spectrometry: data from 1.6 million newborns. Clin Chem (2011) 1.03
Loss of function in phenylketonuria is caused by impaired molecular motions and conformational instability. Am J Hum Genet (2008) 1.02
The Sec-independent function of Escherichia coli YidC is evolutionary-conserved and essential. J Biol Chem (2005) 1.02
The membrane-bound GTPase Guf1 promotes mitochondrial protein synthesis under suboptimal conditions. J Biol Chem (2008) 1.02
Cellular signaling of amino acids towards mTORC1 activation in impaired human leucine catabolism. J Nutr Biochem (2012) 1.01
Screening for congenital adrenal hyperplasia: adjustment of 17-hydroxyprogesterone cut-off values to both age and birth weight markedly improves the predictive value. J Clin Endocrinol Metab (2003) 1.01
Newborn screening for 3-methylcrotonyl-CoA carboxylase deficiency: population heterogeneity of MCCA and MCCB mutations and impact on risk assessment. Hum Mutat (2006) 1.01
Hypermodified fluorescent chlorophyll catabolites: source of blue luminescence in senescent leaves. Angew Chem Int Ed Engl (2010) 1.01
Two novel monothiol glutaredoxins from Saccharomyces cerevisiae provide further insight into iron-sulfur cluster binding, oligomerization, and enzymatic activity of glutaredoxins. Biochemistry (2008) 1.01
Phylogenetic position of Zygogonium ericetorum (zygnematophyceae, charophyta) from a high alpine habitat and ultrastructural characterization of unusual aplanospores. J Phycol (2014) 1.00
Reduced incidence of severe metabolic crisis or death in children with medium chain acyl-CoA dehydrogenase deficiency homozygous for c.985A>G identified by neonatal screening. Mol Genet Metab (2005) 1.00
A novel group of glutaredoxins in the cis-Golgi critical for oxidative stress resistance. Mol Biol Cell (2008) 0.99
Evolution of YidC/Oxa1/Alb3 insertases: three independent gene duplications followed by functional specialization in bacteria, mitochondria and chloroplasts. Biol Chem (2011) 0.99
Fatal neonatal respiratory failure in an infant with congenital hypothyroidism due to haploinsufficiency of the NKX2-1 gene: alteration of pulmonary surfactant homeostasis. Arch Dis Child Fetal Neonatal Ed (2010) 0.99
Structures of chlorophyll catabolites in bananas (Musa acuminata) reveal a split path of chlorophyll breakdown in a ripening fruit. Chemistry (2012) 0.99
Conserved N-terminal negative charges in the Tim17 subunit of the TIM23 translocase play a critical role in the import of preproteins into mitochondria. J Biol Chem (2004) 0.99
Proline residues of transmembrane domains determine the sorting of inner membrane proteins in mitochondria. J Cell Biol (2005) 0.99
Oxidation-driven protein import into mitochondria: Insights and blind spots. Biochim Biophys Acta (2010) 0.98
Atp23 biogenesis reveals a chaperone-like folding activity of Mia40 in the IMS of mitochondria. EMBO J (2012) 0.97
Molecular and immunological characterization of ragweed (Ambrosia artemisiifolia L.) pollen after exposure of the plants to elevated ozone over a whole growing season. PLoS One (2013) 0.95
The Oxa2 protein of Neurospora crassa plays a critical role in the biogenesis of cytochrome oxidase and defines a ubiquitous subbranch of the Oxa1/YidC/Alb3 protein family. Mol Biol Cell (2004) 0.95
Age-related reference values for serum selenium concentrations in infants and children. Clin Chem (2002) 0.94
Cooperation of stop-transfer and conservative sorting mechanisms in mitochondrial protein transport. Curr Biol (2010) 0.94
Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells. Mol Biol Cell (2013) 0.93
Multiplex minisequencing of the 21-hydroxylase gene as a rapid strategy to confirm congenital adrenal hyperplasia. Clin Chem (2002) 0.92
Very high compliance in an expanded MS-MS-based newborn screening program despite written parental consent. Prev Med (2002) 0.92
Insertion of bitopic membrane proteins into the inner membrane of mitochondria involves an export step from the matrix. J Biol Chem (2002) 0.91
Digital image analysis approach for lipid droplet size quantitation of Oil Red O-stained cultured cells. Anal Biochem (2013) 0.91
2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism. Mol Genet Metab (2007) 0.91
Biodiversity of Klebsormidium (streptophyta) from alpine biological soil crusts (alps, tyrol, Austria, and Italy). J Phycol (2015) 0.90
Analysis of biomedical data with multilevel glyphs. BMC Bioinformatics (2014) 0.90
Changing metabolic signatures of amino acids and lipids during the prediabetic period in a pig model with impaired incretin function and reduced β-cell mass. Diabetes (2012) 0.90
Simultaneous quantitative and allele-specific expression analysis with real competitive PCR. BMC Genet (2004) 0.90
Frequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany. Eur J Pediatr (2004) 0.90
Rapid second-tier molecular genetic analysis for congenital adrenal hyperplasia attributable to steroid 21-hydroxylase deficiency. Clin Chem (2004) 0.89
Ribosome-binding proteins Mdm38 and Mba1 display overlapping functions for regulation of mitochondrial translation. Mol Biol Cell (2010) 0.89
Biochemical characterization of dithiol glutaredoxin 8 from Saccharomyces cerevisiae: the catalytic redox mechanism redux. Biochemistry (2009) 0.89
Oxa1-ribosome complexes coordinate the assembly of cytochrome C oxidase in mitochondria. J Biol Chem (2012) 0.89
Mutations in the proenteropeptidase gene are the molecular cause of congenital enteropeptidase deficiency. Am J Hum Genet (2001) 0.87
Role of Pex19p in the targeting of PMP70 to peroxisome. Biochim Biophys Acta (2005) 0.87
The interaction between human PEX3 and PEX19 characterized by fluorescence resonance energy transfer (FRET) analysis. Eur J Cell Biol (2003) 0.87
The rubella virus capsid protein inhibits mitochondrial import. J Virol (2010) 0.87
Targeted inactivation of the murine Abca3 gene leads to respiratory failure in newborns with defective lamellar bodies. Biochem Biophys Res Commun (2007) 0.87
Allele frequencies of the ACTBP2 (=SE33), D18S51, D8S1132, D12S391, D2S1360, D3S1744, D5S2500, D7S1517, D10S2325 and D21S2055 loci in a German population sample. Forensic Sci Int (2005) 0.87