SLC25A15

Summary

Gene Symbol: SLC25A15
Description: solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15
Alias: D13S327, HHH, ORC1, ORNT1, mitochondrial ornithine transporter 1, ornithine transporter 1, solute carrier family 25 member 15
Species: human

Top Publications

  1. pmc Insights into the mutation-induced HHH syndrome from modeling human mitochondrial ornithine transporter-1
    Jing Fang Wang
    Key Laboratory of Systems Biomedicine, Ministry of Education, Shanghai Jiao Tong University, Shanghai, China
    PLoS ONE 7:e31048. 2012
  2. ncbi Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
    S Salvi
    Department of Neurosciences, Pathology, and Radiology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Neurology 57:911-4. 2001
  3. ncbi A novel mutation, P126R, in a Japanese patient with HHH syndrome
    Takeshi Miyamoto
    Department of Inherited Metabolic Disease, National Institute of Neuroscience, NCNP, 4 1 1 Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Pediatr Neurol 26:65-7. 2002
  4. ncbi Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome
    S Salvi
    Molecular Medicine, IRCCS Bambino Gesù, Rome Italy
    Hum Mutat 18:460. 2001
  5. ncbi Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome
    S Tsujino
    Department of Inherited Metabolic Disease, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    Ann Neurol 47:625-31. 2000
  6. ncbi Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter
    J A Camacho
    Institute for Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Nat Genet 22:151-8. 1999
  7. ncbi The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms
    Giuseppe Fiermonte
    Laboratory of Biochemistry and Molecular Biology, Department of Pharmaco Biology, University of Bari, Via Orabona 4, 70125 Bari, Italy
    J Biol Chem 278:32778-83. 2003
  8. doi Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study
    Alessandra Tessa
    Molecular Medicine and Metabolism, Istituto di Ricovero e Cura a Carattere Scientifico Children s Hospital Bambino Gesù, Rome, Italy
    Hum Mutat 30:741-8. 2009
  9. pmc Essential and non-essential DNA replication genes in the model halophilic Archaeon, Halobacterium sp. NRC-1
    Brian R Berquist
    University of Maryland Biotechnology Institute, Center of Marine Biotechnology, Baltimore, Maryland 21202, USA
    BMC Genet 8:31. 2007
  10. doi The human and mouse SLC25A29 mitochondrial transporters rescue the deficient ornithine metabolism in fibroblasts of patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome
    José A Camacho
    Department of Pediatrics, University of California Irvine, Irvine, CA 92697, USA
    Pediatr Res 66:35-41. 2009

Research Grants

  1. Molecular Basis of OTC Deficiency and Related Disorders
    Mendel Tuchman; Fiscal Year: 2005
  2. Structure/Function Studies of DNA Replication Initiation
    James Berger; Fiscal Year: 2007
  3. New Perspectives in Transporter Biology (FASEB Conference)
    Michael Robinson; Fiscal Year: 2005
  4. Optimization of hybrid enzymes for DNA technologies
    Alexei Slesarev; Fiscal Year: 2006
  5. DNA POLYMERASE BETA AND MUTAGENESIS
    Joann B Sweasy; Fiscal Year: 2010
  6. CELL CYCLE OF PLASMODIUM FALCIPARUM
    Debopam Chakrabarti; Fiscal Year: 2003
  7. SLEEP HEART HEALTH STUDY
    Helaine Resnick; Fiscal Year: 2007
  8. DNA replication checkpoints in fission yeast
    GENNARO D URSO; Fiscal Year: 2007
  9. Alzheimer Caregiver Coping: Mental and Physical Health
    Igor Grant; Fiscal Year: 2007
  10. California NeuroAIDS Tissue Network
    Igor Grant; Fiscal Year: 2007

Detail Information

Publications138 found, 100 shown here

  1. pmc Insights into the mutation-induced HHH syndrome from modeling human mitochondrial ornithine transporter-1
    Jing Fang Wang
    Key Laboratory of Systems Biomedicine, Ministry of Education, Shanghai Jiao Tong University, Shanghai, China
    PLoS ONE 7:e31048. 2012
    ..ornithine transporter-1 is reported in coupling with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, which is a rare autosomal recessive disorder...
  2. ncbi Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
    S Salvi
    Department of Neurosciences, Pathology, and Radiology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
    Neurology 57:911-4. 2001
    ..However, all showed signs of pyramidal tract involvement. A broad spectrum of pathogenetic mutations (including missense, nonsense, splice site, insertion, and deletions) were identified in the ORNT1 gene.
  3. ncbi A novel mutation, P126R, in a Japanese patient with HHH syndrome
    Takeshi Miyamoto
    Department of Inherited Metabolic Disease, National Institute of Neuroscience, NCNP, 4 1 1 Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Pediatr Neurol 26:65-7. 2002
    ..Several mutations have been reported in the ORNT1 gene encoding mitochondrial ornithine transporter of patients with this disorder...
  4. ncbi Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome
    S Salvi
    Molecular Medicine, IRCCS Bambino Gesù, Rome Italy
    Hum Mutat 18:460. 2001
    ..the hyperornithinemia, hyperammonemia, and homocitrullinuria (HHH) syndrome were analyzed for mutations in the ORNT1 gene. Seven novel mutations were identified (Q89X, G27R, G190D, R275Q, c.861insG, c.164insA, and IVS5+1G-->A)...
  5. ncbi Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome
    S Tsujino
    Department of Inherited Metabolic Disease, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    Ann Neurol 47:625-31. 2000
    ..We report three novel mutations in the mitochondrial ornithine transporter gene (ORNT1) of Japanese patients with HHH syndrome: a nonsense mutation (R179X) associated with exon skipping and a ..
  6. ncbi Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter
    J A Camacho
    Institute for Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Nat Genet 22:151-8. 1999
    ..We thereby identified such a gene (ORNT1) that maps to 13q14 and whose expression, similar to that of other urea cycle (UC) components, was high in liver ..
  7. ncbi The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms
    Giuseppe Fiermonte
    Laboratory of Biochemistry and Molecular Biology, Department of Pharmaco Biology, University of Bari, Via Orabona 4, 70125 Bari, Italy
    J Biol Chem 278:32778-83. 2003
    Two isoforms of the human ornithine carrier, ORC1 and ORC2, have been identified by overexpression of the proteins in bacteria and by study of the transport properties of the purified proteins reconstituted into liposomes...
  8. doi Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study
    Alessandra Tessa
    Molecular Medicine and Metabolism, Istituto di Ricovero e Cura a Carattere Scientifico Children s Hospital Bambino Gesù, Rome, Italy
    Hum Mutat 30:741-8. 2009
    ..In this study, we collected 16 additional HHH cases and expanded the spectrum of SLC25A15/ORC1 mutations. Eleven novel mutations were identified including six new missense and one microrearrangement...
  9. pmc Essential and non-essential DNA replication genes in the model halophilic Archaeon, Halobacterium sp. NRC-1
    Brian R Berquist
    University of Maryland Biotechnology Institute, Center of Marine Biotechnology, Baltimore, Maryland 21202, USA
    BMC Genet 8:31. 2007
    ..We have developed a tractable genetic system for knockout analysis of genes in the model halophilic archaeon, Halobacterium sp. NRC-1, and used it to determine which DNA replication genes are essential...
  10. doi The human and mouse SLC25A29 mitochondrial transporters rescue the deficient ornithine metabolism in fibroblasts of patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome
    José A Camacho
    Department of Pediatrics, University of California Irvine, Irvine, CA 92697, USA
    Pediatr Res 66:35-41. 2009
    ..urea cycle (UCD) and ornithine degradation pathway caused by mutations in the mitochondrial ornithine transporter (ORNT1)...
  11. ncbi Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder
    José A Camacho
    Department of Pediatrics, University of Oklahoma Health Sciences Center, 975 N E 10th Street, Biomedical Research Center, Room BRC 256, Oklahoma City, OK 73104, USA
    Mol Genet Metab 79:257-71. 2003
    We recently characterized the mitochondrial ornithine transporter (ORNT1), the gene defective in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, a urea cycle disorder...
  12. ncbi Crystal structures of human DNA polymerase beta complexed with DNA: implications for catalytic mechanism, processivity, and fidelity
    H Pelletier
    Department of Chemistry and Biochemistry, University of California, San Diego, La Jolla 92093 0506, USA
    Biochemistry 35:12742-61. 1996
    ..pocket that accommodates a 5'-PO4 end group, the 8-kDa domain also contains a newly discovered helix-hairpin-helix (HhH) motif that binds to DNA in the same way as does a structurally and sequentially homologous HhH motif in the 31-kDa ..
  13. pmc A genetic screen for replication initiation defective (rid) mutants in Schizosaccharomyces pombe
    Alexandra M Locovei
    Department of Molecular and Cellular Phamacology, University of Miami School of Medicine PO Box 016189, Miami, FL 33140, USA
    Cell Div 5:20. 2010
    ..Our screen identified two mutants, rid1-1 and rid2-1, that are defective in Orc1 and Mcm4, respectively...
  14. pmc Genetic and physical mapping of DNA replication origins in Haloferax volcanii
    Cedric Norais
    Institut de Genetique et Microbiologie, Universite Paris Sud, Orsay, France
    PLoS Genet 3:e77. 2007
    ..Genes for the initiator protein Cdc6/Orc1, which are commonly located adjacent to archaeal origins of DNA replication, are found on all replicons except ..
  15. doi RNA interference targeting ORC1 gene suppresses the proliferation of vascular smooth muscle cells in rats
    Mao Qin Shu
    Department of Cardiology, Southwest Hospital, The Third Military Medical University, Chongqing 400038, China
    Exp Mol Pathol 84:206-12. 2008
    ..The largest subunit of the origin recognition complex (ORC), ORC1, plays a critical role during the initiation of DNA replication in eukaryotes...
  16. pmc Genomewide and biochemical analyses of DNA-binding activity of Cdc6/Orc1 and Mcm proteins in Pyrococcus sp
    Fujihiko Matsunaga
    Institut de Genetique et Microbiologie, UMR8621, Bat 409, Universite Paris Sud, 91405 Orsay Cedex, France
    Nucleic Acids Res 35:3214-22. 2007
    ..We report here that the binding of Cdc6/Orc1 to a 5 kb region containing oriC in vivo was highly specific both in exponential and stationary phases, by means of ..
  17. doi Genetic variation in the urea cycle: a model resource for investigating key candidate genes for common diseases
    Sabrina Mitchell
    Center for Human Genetics Research, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA
    Hum Mutat 30:56-60. 2009
    ..boundaries of the five key urea cycle enzymes, NAGS, and two solute carrier transporter genes (SLC25A13 and SLC25A15) for sequence alterations using single-stranded conformational polymorphism (SSCP) analysis and high-resolution ..
  18. doi Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failure
    A A Mhanni
    Department of Pediatrics and Child Health, University of Manitoba, Canada
    J Pediatr Gastroenterol Nutr 46:312-5. 2008
    ..Molecular analysis of the SLC25A15 gene showed that the two patients were homozygous for the common French Canadian mutation (F188Delta)...
  19. ncbi Octahedral tilting in Pb-based relaxor ferroelectrics at high pressure
    Bernd J Maier
    Department Geowissenschaften, , Grindelallee 48, 20146 Hamburg, Germany
    Acta Crystallogr B 66:280-91. 2010
    ..Synchrotron single-crystal X-ray diffraction data show that the intensity of hhh peaks, h = 2n + 1, does not change with pressure...
  20. pmc Crystal structure of an archaeal Ski2p-like protein from Pyrococcus horikoshii OT3
    Xiaodong Zhang
    Laboratory of Structural Biology, Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 812 8581, Japan
    Protein Sci 17:136-45. 2008
    ..C1 and C2) fold into alpha-helical structures containing a winged helix (WH)-fold and helix-hairpin-helix (HhH)-fold, respectively...
  21. ncbi Mammary sensitivity to protein restriction and re-alimentation
    M G Goodwill
    Institute of Ecology and Resource Management, University of Edinburgh
    Br J Nutr 76:423-34. 1996
    ..Lactational dietary treatments were the supply ad libitum of either diet H (HHH) or diet L (LLL) for the first 12 d of lactation, or diet L transferring to diet H on either day 6 (LHH) or 9 (LLH) ..
  22. ncbi Functional changes in a novel uracil-DNA glycosylase determined by mutational analyses
    E K Im
    Yonsei Research Institute of Aging Science, Yonsei University, Seoul 120 749, South Korea
    Mikrobiologiia 77:644-50. 2008
    ..Methanococcus jannaschii UDG (MjUDG), a novel monofunctional glycosylase, contains a helix-hairpin-helix (HhH) motif and Gly/Pro rich loop (GPD region), which is important for catalytic activity; it shares these features with ..
  23. pmc Novel adhesin from Pasteurella multocida that binds to the integrin-binding fibronectin FnIII9-10 repeats
    Lisa M Mullen
    Division of Microbial Diseases, Eastman Dental Institute, University College London, 256 Gray s Inn Road, London WC1X 8LD, United Kingdom
    Infect Immun 76:1093-104. 2008
    ..Bioinformatics analysis suggests that the PM1665 protein contains two helix-hairpin-helix (HhH) motifs, and truncation mutation studies have identified the binding site in the protein as a combination of these ..
  24. ncbi Intra-arterial verapamil-induced seizures: case report and review of the literature
    Franklin D Westhout
    Department of Neurological Surgery, University of California Irvine School of Medicine, Orange, CA 92868, USA
    Surg Neurol 67:483-6; discussion 486. 2007
    ..Seizures occurring from IA infusion of verapamil are rare...
  25. ncbi Structure and activity of a thermostable thymine-DNA glycosylase: evidence for base twisting to remove mismatched normal DNA bases
    Clifford D Mol
    Department of Molecular Biology MB4, Skaggs Institute for Chemical Biology, 10550 North Torrey Pines Rd, La Jolla, CA 92037, USA
    J Mol Biol 315:373-84. 2002
    ..repair can be initiated by a specific mismatch glycosylase (MIG) that is homologous to the helix-hairpin-helix (HhH) DNA repair enzymes. Here, we present a 2...
  26. doi Orotic acid quantification in dried blood spots and biological fluids by hydrophilic interaction liquid chromatography tandem mass spectrometry
    Oceania D'Apolito
    Department of Biomedical Sciences, University of Foggia, Foggia, Italy
    J Sep Sci 33:966-73. 2010
    ..A patient with hyperammonemia-hyperornithinemia-homocitrullinemia (HHH) syndrome presented a urinary ORA level of 9.1 mmol/mol of creatinine...
  27. ncbi Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome
    José A Camacho
    Department of Pediatrics, Division of Human Genetics, University of California, Irvine, CA 92697 3940, USA
    Pediatr Res 60:423-9. 2006
    ..a disorder of the urea cycle and ornithine degradation pathway, who have the same novel ornithine transporter (ORNT1) genotype (T32R) but a variable phenotype...
  28. pmc DNA replication and the GINS complex: localization on extended chromatin fibers
    Stephanie M Cohen
    Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA
    Epigenetics Chromatin 2:6. 2009
    ..RESULTS: Using immunofluorescence microscopy we were able to visualize ORC1, ORC2, PCNA, and GINS complex proteins Psf1 and Psf2 bound to extended chromatin fibers...
  29. pmc The HhH2/NDD domain of the Drosophila Nod chromokinesin-like protein is required for binding to chromosomes in the oocyte nucleus
    Wei Cui
    Stowers Institute for Medical Research, 1000 E 50th Street, Kansas City, MO 64110, USA
    Genetics 171:1823-35. 2005
    ..The second putative DNA-binding domain, denoted HhH(2)/NDD, is a helix-hairpin-helix(2)/Nod-like DNA-binding domain...
  30. pmc Trypanosome prereplication machinery contains a single functional orc1/cdc6 protein, which is typical of archaea
    Patrícia Diogo de Melo Godoy
    Laboratorio de Parasitologia, Instituto Butantan, Av Vital Brasil, 1500, São Paulo 05503900, Brazil
    Eukaryot Cell 8:1592-603. 2009
    ..the replication origin is recognized by the heterohexamer origin recognition complex (ORC) containing six proteins, Orc1 to Orc6, while in members of the domain Archaea, the replication origin is recognized by just one protein, Orc1/..
  31. ncbi Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia
    J Muhling
    Department of Anaesthesiology and Intensive Care Medicine, Justus Liebig University Giessen, Federal Republic of Germany
    Amino Acids 21:303-18. 2001
    ..combination of increased plasma ornithine concentrations, postprandial hyperammonemia, and homocitrullinuria (HHH-syndrome)...
  32. doi Mutational studies of Pa-AGOG DNA glycosylase from the hyperthermophilic crenarchaeon Pyrobaculum aerophilum
    Gondichatnahalli M Lingaraju
    Biomolecular Research, Paul Scherrer Institut, CH 5232 Villigen, Switzerland
    DNA Repair (Amst) 8:857-64. 2009
    ..glycosylase, Pa-AGOG (Archaeal GO DNA glycosylase) which has become the founding member of a new family within the HhH-GPD superfamily of DNA glycosylases based on unique structural and functional characteristics...
  33. pmc The cyclin A centrosomal localization sequence recruits MCM5 and Orc1 to regulate centrosome reduplication
    Rebecca L Ferguson
    Howard Hughes Medical Institute and Department of Pharmacology, University of Colorado School of Medicine, Aurora, CO 80045, USA
    J Cell Sci 123:2743-9. 2010
    ..Previous studies have demonstrated a role for the DNA replication factors MCM5 and Orc1 in preventing centrosome reduplication...
  34. doi HHH regime for arteritis secondary to TB meningitis: a prospective randomized study
    Arunodaya R Gujjar
    Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India
    Neurocrit Care 10:313-7. 2009
    ..Few treatment regimes specifically address this condition. Hypervolemia-hypertension-hemodilution (HHH) regime is known to be effective for treatment of vasospasm complicating subarachnoid hemorrhage...
  35. pmc Binding of AlF-C, an Orc1-binding transcriptional regulator, enhances replicator activity of the rat aldolase B origin
    Hiroyuki Minami
    Cryobiosystem Research Center, Iwate University, Ueda, Morioka, Iwate 020 8550, Japan
    Mol Cell Biol 26:8770-80. 2006
    ..Mutation of site C in the rat origin, which binds an Orc1-binding protein AlF-C in vitro, resulted in a significant reduction of the replicator activity in the mouse cells...
  36. ncbi Health consequences of Alzheimer's caregiving transitions: effects of placement and bereavement
    Igor Grant
    Department of Psychiatry, University of California, San Diego, La Jolla, California 92093 0680, USA
    Psychosom Med 64:477-86. 2002
    ....
  37. doi Cdc6/Orc1 from Pyrococcus furiosus may act as the origin recognition protein and Mcm helicase recruiter
    Masaki Akita
    Graduate School of Bioresource and Bioenvironmental Sciences, Kyushu University, 6 10 1 Hakozaki, Higashi ku, Fukuoka, Fukuoka 812 8581, Japan
    Genes Cells 15:537-52. 2010
    Archaea have one or more Cdc6/Orc1 proteins, which share sequence similarities with eukaryotic Cdc6 and Orc1...
  38. ncbi Is hereditary hemochromatosis a balanced polymorphism: an analysis of family size among hemochromatosis heterozygotes
    R L Nelson
    Department of Surgery, College of Medicine, University of Illinois at Chicago, 1740 West Taylor Street, Room 2204, m c 957, Chicago, Illinois 60612, USA
    Hepatogastroenterology 48:523-6. 2001
    ..Evidence for this was sought by assessing reproductive success among hemochromatosis heterozygotes through determining their family size and incidence of miscarriage when compared to nongene carriers...
  39. ncbi Safety of intrathecal sodium nitroprusside for the treatment and prevention of refractory cerebral vasospasm and ischemia in humans
    J E Thomas
    Department of Neurological Surgery, Division of Cerebrovascular Surgery, Thomas Jefferson University, Wills Eye Hospital, Philadelphia, PA, USA
    Stroke 30:1409-16. 1999
    ..The mechanism of DCV remains unknown. A new treatment for refractory DCV using intrathecally delivered sodium nitroprusside and results in 21 patients is reported...
  40. doi Localized melting of duplex DNA by Cdc6/Orc1 at the DNA replication origin in the hyperthermophilic archaeon Pyrococcus furiosus
    Fujihiko Matsunaga
    Department of Genetic Resources Technology, Graduate School of Bioresource and Bioenvironmental Sciences, Kyushu University, 6 10 1 Hakozaki, Higashi ku, Fukuoka, Fukuoka 812 8581, Japan
    Extremophiles 14:21-31. 2010
    ..Although recent studies in Archaea defined the origin of DNA replication (oriC) and the Cdc6/Orc1 homolog as an origin recognition protein, the location and mechanism of duplex opening have remained unclear...
  41. ncbi The archaeal cell cycle: current issues
    Rolf Bernander
    Department of Molecular Evolution, Evolutionary Biology Center, Uppsala University, Norbyvagen 18C, SE 752 36 Uppsala, Sweden
    Mol Microbiol 48:599-604. 2003
    The recently discovered structural similarities between the archaeal Orc1/Cdc6 and bacterial DnaA initiator proteins for chromosome replication have exciting implications for cell cycle regulation...
  42. pmc Pa-AGOG, the founding member of a new family of archaeal 8-oxoguanine DNA-glycosylases
    Alessandro A Sartori
    Institute of Molecular Cancer Research, University of Zurich, August Forel Strasse 7, Ch 8008 Zurich, Switzerland
    Nucleic Acids Res 32:6531-9. 2004
    ..a helix-hairpin-helix motif and a glycine/proline-rich sequence followed by an absolutely conserved aspartate (HhH-GPD motif). Interestingly, although the P...
  43. pmc Chromosome replication dynamics in the archaeon Sulfolobus acidocaldarius
    Iain G Duggin
    Medical Research Council Cancer Cell Unit, Hutchison Medical Research Council Research Centre, Hills Road, Cambridge, CB2 0XZ, United Kingdom
    Proc Natl Acad Sci U S A 105:16737-42. 2008
    ..Analysis of the 3 Orc1/Cdc6 initiator proteins showed a uniformity of cellular abundance and origin binding throughout the cell cycle...
  44. pmc Transcriptional silencing functions of the yeast protein Orc1/Sir3 subfunctionalized after gene duplication
    Meleah A Hickman
    Biochemistry Department, Institute for Genome Sciences and Policy, Program in Genetics and Genomics, Duke University, Durham, NC 27708, USA
    Proc Natl Acad Sci U S A 107:19384-9. 2010
    ..The largest subunit of ORC, Orc1, is particularly interesting because it contains a nucleosome-binding BAH domain and because it gave rise to Sir3, ..
  45. pmc Elaboration, diversification and regulation of the Sir1 family of silencing proteins in Saccharomyces
    Jennifer E G Gallagher
    Department of Molecular and Cell Biology, California Institute of Quantitative Biosciences, University of California, Berkeley, CA 94720 3220, USA
    Genetics 181:1477-91. 2009
    ..at HML and HMR via its ORC interacting region (OIR), which binds the bromo adjacent homology (BAH) domain of Orc1. Zygosaccharomyces rouxii, which diverged from Saccharomyces after the appearance of the silent mating cassettes, ..
  46. ncbi A DNA glycosylase from Pyrobaculum aerophilum with an 8-oxoguanine binding mode and a noncanonical helix-hairpin-helix structure
    Gondichatnahalli M Lingaraju
    Biomolecular Research, Paul Scherrer Institut, CH 5232 Villigen, Switzerland
    Structure 13:87-98. 2005
    ..are identified that confirm Pa-AGOG to be the founding member of a functional class within the helix-hairpin-helix (HhH) superfamily of DNA repair enzymes...
  47. ncbi Deoxyribose phosphate excision by the N-terminal domain of the polymerase beta: the mechanism revisited
    J A Feng
    Institute for Cancer Research, Fox Chase Cancer Center, Philadelphia, Pennsylvania 19111, USA
    Biochemistry 37:9605-11. 1998
    ..This mechanism focuses on a catalytic groove near the helix-hairpin-helix (HhH) motif of the 8 kDa domain...
  48. ncbi Proteomic analysis of human O6-methylguanine-DNA methyltransferase by affinity chromatography and tandem mass spectrometry
    Suryakant K Niture
    Department of Pharmaceutical Sciences, School of Pharmacy, Texas Tech University Health Sciences Center, Amarillo, TX 79106, USA
    Biochem Biophys Res Commun 337:1176-84. 2005
    ..proteins with diverse functions including those involved in DNA replication and repair (MCM2, PCNA, ORC1, DNA polymerase delta, MSH-2, and DNA-dependent protein kinase), cell cycle progression (CDK1, cyclin B, CDK2, CDC7,..
  49. ncbi Over-expression of human DNA polymerase lambda in E. coli and characterization of the recombinant enzyme
    Noriko Shimazaki
    Department of Applied Biological Science, Faculty of Science and Technology, Science University of Tokyo, Noda, Chiba 278 8510, Japan
    Genes Cells 7:639-51. 2002
    ..a nuclear localization signal (NLS), a BRCA1-C terminal (BRCT) domain, a proline-rich region, helix-hairpin-helix (HhH) and pol X motifs...
  50. ncbi Hormonal induction of hepatic mitochondrial ornithine/citrulline transporter mRNA
    Sidney M Morris
    Department of Molecular Genetics and Biochemistry, University of Pittsburgh School of Medicine, W1255 Biomedical Science Tower, PA 15206, USA
    Biochem Biophys Res Commun 294:749-52. 2002
    ..transport of ornithine and citrulline across the mitochondrial membrane by the ornithine/citrulline antiporter ORNT1. Expression of the urea cycle enzymes can change dramatically in response to hormones, but it is not known whether ..
  51. ncbi The essential role of Saccharomyces cerevisiae CDC6 nucleotide-binding site in cell growth, DNA synthesis, and Orc1 association
    B Wang
    Department of Pediatrics, University of Southern California, Los Angeles, California 90027, USA
    J Biol Chem 274:8291-8. 1999
    ..the molecular mechanism of nucleotide-binding function, we have demonstrated that the Cdc6 protein associates with Orc1 in vitro and in vivo...
  52. ncbi Conformational changes induced by nucleotide binding in Cdc6/ORC from Aeropyrum pernix
    Martin R Singleton
    Cancer Research UK Clare Hall Laboratories, The London Research Institute, Blanche Lane, South Mimms, Potters Bar, Herts EN6 3LD, UK
    J Mol Biol 343:547-57. 2004
    ..suggests the existence of at least two subfamilies of these proteins, for which we propose the nomenclature ORC1 and ORC2...
  53. ncbi Characterization of the metal ion binding helix-hairpin-helix motifs in human DNA polymerase beta by X-ray structural analysis
    H Pelletier
    Department of Chemistry and Biochemistry, University of California, San Diego, La Jolla 92093 0506, USA
    Biochemistry 35:12778-87. 1996
    ..beta (pol beta) occurs primarily through two structurally and sequentially homologous helix-hairpin-helix (HhH) motifs, one in the fingers subdomain and the other in the 8-kDa domain [Pelletier, H., Sawaya, M. R., Wolfle, W...
  54. pmc Structure-guided mutational analysis of the OB, HhH, and BRCT domains of Escherichia coli DNA ligase
    Li Kai Wang
    Molecular Biology Program, Sloan Kettering Institute, New York, New York 10021, USA
    J Biol Chem 283:23343-52. 2008
    ..and oligonucleotide-binding (OB) domains is linked via a tetracysteine zinc finger to distal helix-hairpin-helix (HhH) and BRCT (BRCA1-like C-terminal) domains...
  55. ncbi Novel splicing variant of mouse Orc1 is deficient in nuclear translocation and resistant for proteasome-mediated degradation
    Yasuyuki Miyake
    Cellular Physiology Laboratory, RIKEN Discovery Research Institute, Wako, Saitama 351 0198, Japan
    J Biol Chem 280:12643-52. 2005
    ..of the mammalian ORC, we cloned ORC subunit cDNAs from mouse NIH3T3 cells and found novel variant forms of Orc1, Orc2, and Orc3 each derived from alternative RNA splicing...
  56. pmc HLA-A2.1-restricted education and cytolytic activity of CD8(+) T lymphocytes from beta2 microglobulin (beta2m) HLA-A2.1 monochain transgenic H-2Db beta2m double knockout mice
    S Pascolo
    Institut Pasteur, Département SIDA Rétrovirus, Unité d Immunité Cellulaire Antivirale, 75724 Paris Cedex 15, France
    J Exp Med 185:2043-51. 1997
    ..beta2m) is covalently linked to the NH2 terminus of the heavy chain by a 15- amino acid long peptide: HHH, entirely human, HHD, with the mouse H-2Db alpha3, transmembrane, and cytoplasmic domains, and MHD, homologous to ..
  57. pmc Selective instability of Orc1 protein accounts for the absence of functional origin recognition complexes during the M-G(1) transition in mammals
    D A Natale
    National Institute of Child Health and Human Development, Building 6, Room 3A02, National Institutes of Health, Bethesda, MD 20892 2753, USA
    EMBO J 19:2728-38. 2000
    ..the time when hamster origin recognition complexes (ORCs) became functional was related to the time when Orc1, Orc2 and Mcm3 proteins became stably bound to hamster chromatin...
  58. ncbi Functional differentiation and cooperative interaction between two eukaryote-like archaeal Orc1/Cdc6 proteins on the replication origin
    Pei Xia Jiang
    Center for Proteomics Research, National Key Laboratory of Agricultural Microbiology, College of Life Science and Technology, Huazhong Agricultural University, Wuhan 430070, China
    Biochem Biophys Res Commun 364:945-51. 2007
    The DNA replication apparatus of archaea represents a core version of that in eukaryotes. Archaeal Orc1/Cdc6s can be an integral component in the replication machineries cooperatively regulating DNA replication...
  59. pmc The Methanosarcina barkeri genome: comparative analysis with Methanosarcina acetivorans and Methanosarcina mazei reveals extensive rearrangement within methanosarcinal genomes
    Dennis L Maeder
    Center of Marine Biotechnology, University of Maryland Biotechnology Institute, 701 E Pratt Street, Baltimore, MD 21202, USA
    J Bacteriol 188:7922-31. 2006
    ..A cryptic 36-kbp plasmid sequence that contains an orc1 gene flanked by a presumptive origin of replication consisting of 38 tandem repeats of a 143-nucleotide motif was ..
  60. pmc Three replication origins in Sulfolobus species: synchronous initiation of chromosome replication and asynchronous termination
    Magnus Lundgren
    Department of Molecular Evolution, Evolutionary Biology Center, Uppsala University, Norbyvagen 18C, SE 752 36 Uppsala, Sweden
    Proc Natl Acad Sci U S A 101:7046-51. 2004
    ..Two of the three replication origins in each species were located in the vicinity of a cdc6/orc1 replication initiation gene, whereas no known replication-associated gene could be identified near the third origin ..
  61. pmc Role for Cdk1 (Cdc2)/cyclin A in preventing the mammalian origin recognition complex's largest subunit (Orc1) from binding to chromatin during mitosis
    Cong jun Li
    Growth Biology Laboratory, Animal and Natural Resources Institute, Agricultural Research Service, U S Department of Agriculture, Beltsville, Maryland 20705, USA
    Mol Cell Biol 24:5875-86. 2004
    ..In proliferating mammalian cells, ORC activity appears to be regulated by reducing the affinity of the Orc1 subunit for chromatin during S phase and then preventing reformation of a stable ORC-chromatin complex until ..
  62. ncbi Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in mice
    Laila Begum
    Department of Biochemistry, Faculty of Medicine, Kagoshima University, 8 35 1 Sakuragaoka, 890 8520, Kagoshima, Japan
    Biochim Biophys Acta 1574:283-92. 2002
    ..of mouse aspartate/glutamate carrier (AGC) genes, Slc25a13 and Slc25a12, and an ornithine transporter gene, Ornt1, in relation to urea cycle enzyme genes, carbamoylphosphate synthetase I (CPS) and argininosuccinate synthetase (..
  63. ncbi Screening for mutations in the GJB3 gene in Brazilian patients with nonsyndromic deafness
    Fabiana Alexandrino
    CBMEG Human Genetics Laboratory, Campinas, SP, Brazil
    J Appl Genet 45:249-54. 2004
    ..uia.ac.be/dnalab/hhh/)...
  64. pmc ORC, MCM, and histone hyperacetylation at the Kaposi's sarcoma-associated herpesvirus latent replication origin
    William Stedman
    The Wistar Institute, 3601 Spruce Street, Philadelphia, PA 19104 4268, USA
    J Virol 78:12566-75. 2004
    ..ORC2 bound TR specifically and identified the histone acetyltransferase HBO1 (histone acetyltransferase binding to ORC1) as a potential TR binding protein...
  65. ncbi Characterization of the origin recognition complex (ORC) from a higher plant, rice (Oryza sativa L.)
    Yoko Mori
    Department of Applied Biological Science, Faculty of Science and Technology, Science University of Tokyo, 2641 Yamazaki, Noda shi, Chiba ken 278 8510, Japan
    Gene 353:23-30. 2005
    ..The protein is composed of six subunits (ORC1 to 6) in animals and yeasts. Our knowledge of the ORC protein in plants is, however, much less complete...
  66. doi Non-productive DNA damage binding by DNA glycosylase-like protein Mag2 from Schizosaccharomyces pombe
    Suraj Adhikary
    Department of Biological Sciences, Vanderbilt University, Nashville, TN 37232, USA
    DNA Repair (Amst) 12:196-204. 2013
    Schizosaccharomyces pombe contains two paralogous proteins, Mag1 and Mag2, related to the helix-hairpin-helix (HhH) superfamily of alkylpurine DNA glycosylases from yeast and bacteria...
  67. pmc Mechanistic assessment of DNA ligase as an antibacterial target in Staphylococcus aureus
    Steven D Podos
    Achillion Pharmaceuticals, New Haven, Connecticut, USA
    Antimicrob Agents Chemother 56:4095-102. 2012
    ..catalytic adenylation domain and 19 in the downstream oligonucleotide-binding (OB) fold and helix-hairpin-helix (HhH) domains...
  68. pmc Trypanosoma brucei Orc1 is essential for nuclear DNA replication and affects both VSG silencing and VSG switching
    Imaan Benmerzouga
    Center for Gene Regulation in Health and Disease, Department of Biological, Geological, and Environmental Sciences, Cleveland State University, Cleveland, OH 44115, USA
    Mol Microbiol 87:196-210. 2013
    ..An Orc1/Cdc6 homologue has been identified in T...
  69. pmc Molecular characterization of NAD+-dependent DNA ligase from Wolbachia endosymbiont of lymphatic filarial parasite Brugia malayi
    Nidhi Shrivastava
    Division of Parasitology, Central Drug Research Institute, Lucknow, Uttar Pradesh, India
    PLoS ONE 7:e41113. 2012
    ..the eubacterial NAD(+)-dependent DNA ligases such as N-terminal adenylation domain, OB fold, helix-hairpin-helix (HhH) and BRCT domain except zinc-binding tetracysteine domain...
  70. pmc Specificity and function of archaeal DNA replication initiator proteins
    Rachel Y Samson
    Sir William Dunn School of Pathology, Oxford University, South Parks Road, Oxford OX1 3RE, UK
    Cell Rep 3:485-96. 2013
    ..While two origins are dependent on archaeal homologs of eukaryal Orc1 and Cdc6, the third origin is instead reliant on an archaeal Cdt1 homolog...
  71. pmc In the absence of ATPase activity, pre-RC formation is blocked prior to MCM2-7 hexamer dimerization
    Cecile Evrin
    DNA replication Group, MRC Clinical Sciences Centre, Imperial College, London W12 0NN, UK
    Nucleic Acids Res 41:3162-72. 2013
    ..Moreover, we show that Orc1 ATP hydrolysis is not involved in helicase loading or in releasing ORC from loaded MCM2-7...
  72. doi Long-term follow-up of four patients affected by HHH syndrome
    Sook Z Kim
    Korea Genetic Research Center, Cheong Ju, Heung Deok Gu, Republic of Korea
    Clin Chim Acta 413:1151-5. 2012
    In hyperornithinemia-hyperammonemia-homocitrullinemia (HHH) syndrome, impaired ornithine transport across the mitochondrial membrane causes ornithine accumulation in cytoplasm...
  73. doi Creatine metabolism in urea cycle defects
    Sara Boenzi
    Division of Metabolism and Research Unit of Metabolic Biochemistry, Bambino Gesu Children s Hospital, IRCCS, Piazza S Onofrio 4, 00165, Rome, Italy
    J Inherit Metab Dis 35:647-53. 2012
    ..e., OTC, ASS, ASL deficiencies, HHH syndrome and lysinuric protein intolerance)...
  74. pmc Cdc6-induced conformational changes in ORC bound to origin DNA revealed by cryo-electron microscopy
    Jingchuan Sun
    Biology Department, Brookhaven National Laboratory, Upton, NY 11973, USA
    Structure 20:534-44. 2012
    ..The six subunits of ORC are arranged as Orc1:Orc4:Orc5:Orc2:Orc3, with Orc6 binding to Orc2...
  75. pmc Substrate specificity of the two mitochondrial ornithine carriers can be swapped by single mutation in substrate binding site
    Magnus Monné
    Department of Pharmaco Biology, Laboratory of Biochemistry and Molecular Biology, University of Bari, Via E Orabona 4, 70125 Bari, Italy
    J Biol Chem 287:7925-34. 2012
    ..Here we have characterized mutations of the proposed substrate binding site in the human ornithine carriers ORC1 and ORC2 by carrying out transport assays with a set of different substrates...
  76. pmc Human origin recognition complex is essential for HP1 binding to chromatin and heterochromatin organization
    Supriya G Prasanth
    Cold Spring Harbor Laboratory, Cold Spring Harbor, NY 11724, USA
    Proc Natl Acad Sci U S A 107:15093-8. 2010
    ..Fluorescent bleaching studies indicate that multiple subcomplexes of ORC exist at heterochromatin, with Orc1 stably associating with heterochromatin in G1 phase, whereas other ORC subunits have transient interactions ..
  77. ncbi Replication factors MCM2 and ORC1 interact with the histone acetyltransferase HBO1
    T W Burke
    Department of Genetics, Howard Hughes Medical Institute, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Biol Chem 276:15397-408. 2001
    ..One of the proteins we identified is identical to the ORC1-interacting protein termed HBO1...
  78. pmc The conserved bromo-adjacent homology domain of yeast Orc1 functions in the selection of DNA replication origins within chromatin
    Philipp Müller
    Department of Biomolecular Chemistry, University of Wisconsin Madison, Madison, Wisconsin 53706, USA
    Genes Dev 24:1418-33. 2010
    ..This study focused on the role of the conserved N-terminal bromo-adjacent homology domain of yeast Orc1 (Orc1BAH). Recent studies indicate that BAH domains are chromatin-binding modules...
  79. pmc Estrogen receptor α (ERα) mediates 17β-estradiol (E2)-activated expression of HBO1
    Wen zhong Wang
    Department of Biochemistry and Molecular Biology, Shanghai Medical College of Fudan University, Shanghai, 200032, China
    J Exp Clin Cancer Res 29:140. 2010
    HBO1 (histone acetyltransferase binding to ORC1) is a histone acetyltransferase (HAT) which could exert oncogenic function in breast cancer...
  80. ncbi An evolutionary analysis of the helix-hairpin-helix superfamily of DNA repair glycosylases
    Dee R Denver
    Department of Biology, Indiana University, USA
    Mol Biol Evol 20:1603-11. 2003
    The helix-hairpin-helix (HhH) superfamily of base excision repair DNA glycosylases is composed of multiple phylogenetically diverse enzymes that are capable of excising varying spectra of oxidatively and methyl-damaged bases...
  81. pmc Involvement of RAD9-dependent damage checkpoint control in arrest of cell cycle, induction of cell death, and chromosome instability caused by defects in origin recognition complex in Saccharomyces cerevisiae
    Keiichi Watanabe
    Department of Molecular Biology, Graduate School of Biological Sciences, Nara Institute of Science and Technology NAIST, Ikoma, Nara 630 0101, Japan
    Eukaryot Cell 1:200-12. 2002
    ..The cell cycle of orc1-4/orc1-4 temperature-sensitive mutant was arrested at the G2/M boundary, after several rounds of cell division at ..
  82. ncbi The multidomain structure of Orc1p reveals similarity to regulators of DNA replication and transcriptional silencing
    S P Bell
    Cold Spring Harbor Laboratory, New York 11724, USA
    Cell 83:563-8. 1995
    ..Here we report the cloning of the genes encoding the 120 kDa (ORC1), 62 kDa (ORC3), and 56 kDa (ORC4) subunits of ORC and the reconstitution of the complete complex after expression ..
  83. ncbi Assessing individual bioequivalence with high-order cross-over designs: a unified procedure
    Francis C Hsuan
    Temple University, Philadelphia, PA 19122, USA
    Stat Med 22:2847-60. 2003
    ..The recommended approach to the analysis is to follow the methodology of Hyslop, Hsuan and Holder (HHH), based on a linear mixed model...
  84. doi Quantitation of orotic acid in urine using isotope dilution-selected ion gas chromatography-mass spectrometry
    Jie Chen
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Methods Mol Biol 603:445-51. 2010
    ..including ornithine transcarbamylase (OTC) deficiency, and the hyperornithinemia-hyperammonemia-homocitrullinemia (HHH) syndrome. Low levels of orotic acid are observed in carbamylphosphate synthetase (CPS) defects...
  85. pmc Non-specific DNA binding interferes with the efficient excision of oxidative lesions from chromatin by the human DNA glycosylase, NEIL1
    Ian D Odell
    Department of Microbiology and Molecular Genetics, University of Vermont, Burlington, VT 05405, United States
    DNA Repair (Amst) 9:134-43. 2010
    ..We reported recently that the human DNA glycosylase hNTH1 (human Endonuclease III), a member of the HhH GpG superfamily of glycosylases, can excise thymine glycol lesions from nucleosomes without requiring or inducing ..
  86. ncbi The mitochondrial transporter family (SLC25): physiological and pathological implications
    Ferdinando Palmieri
    Department of Pharmaco Biology, Laboratory of Biochemistry and Molecular Biology, University of Bari, Via Orabona 4, 70125, Bari, Italy
    Pflugers Arch 447:689-709. 2004
    ..g. hyperornithinaemia, hyperammonaemia and homocitrullinuria (HHH) syndrome and type II citrullinemia...
  87. pmc An episomal mammalian replicon: sequence-independent binding of the origin recognition complex
    Daniel Schaarschmidt
    Department of Biology, Universitat Konstanz, Konstanz, Germany
    EMBO J 23:191-201. 2004
    ..immunoprecipitations and quantitative polymerase chain reaction analysis revealed that, in G1-phase cells, Orc1 and Orc2, as well as Mcm3, another component of the prereplication complex, are bound to multiple sites on the ..
  88. doi Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats
    Carolina Maso Viegas
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
    Brain Res 1291:102-12. 2009
    ..acid (Oro) is the biochemical hallmark of patients affected by hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, a disorder clinically characterized by neurological symptoms, whose pathophysiology is practically ..
  89. doi Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
    Carolina Maso Viegas
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
    Brain Res 1369:235-44. 2011
    Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is an autosomal recessive disorder caused by a defect in the mitochondrial ornithine transporter, leading to accumulation of ornithine (Orn), homocitrulline (Hcit) and ..
  90. ncbi Subsets of human origin recognition complex (ORC) subunits are expressed in non-proliferating cells and associate with non-ORC proteins
    K C Thome
    Division of Molecular Oncology, Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 275:35233-41. 2000
    ..Finally, all ORC subunits remain consistently nuclear, and Orc2 is consistently phosphorylated through all stages of the cell cycle, whereas Orc1 is selectively phosphorylated in mitosis.
  91. pmc Novel DNA binding motifs in the DNA repair enzyme endonuclease III crystal structure
    M M Thayer
    Scripps Research Institute, Department of Molecular Biology MB4, La Jolla, CA 92037, USA
    EMBO J 14:4108-20. 1995
    ..Helix-hairpin-helix (HhH) and [4Fe-4S] cluster loop (FCL) motifs, which we have named for their secondary structure, bracket the cleft ..
  92. pmc Expression of the HsOrc1 gene, a human ORC1 homolog, is regulated by cell proliferation via the E2F transcription factor
    K Ohtani
    Department of Genetics, Howard Hughes Medical Institute, Duke University Medical Center, Durham, North Carolina 27710, USA
    Mol Cell Biol 16:6977-84. 1996
    ..Expression of the human Orc1 gene (HsOrc1) is low in quiescent cells, and it is then dramatically induced upon stimulation of cell growth...
  93. pmc Human CDC6/Cdc18 associates with Orc1 and cyclin-cdk and is selectively eliminated from the nucleus at the onset of S phase
    P Saha
    Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Mol Cell Biol 18:2758-67. 1998
    ..with human PCNA, we identified and cloned a human protein (hCdc18) homologous to yeast CDC6/Cdc18 and human Orc1. Unlike yeast, in which the rapid and total destruction of CDC6/Cdc18 protein in S phase is a central feature of ..
  94. ncbi Regulation of cell growth-dependent expression of mammalian CDC6 gene by the cell cycle transcription factor E2F
    K Ohtani
    Human Gene Sciences Center, Graduate School of Dentistry, Tokyo Medical and Dental University, Japan
    Oncogene 17:1777-85. 1998
    ..or HsOrc1 failed to induce DNA synthesis unlike overexpression of E2F1, even though E2F1 induced both Cdc6 and Orc1, suggesting that E2F may regulate the expression of another gene(s), besides Cdc6 and Orc1, required for induction ..
  95. ncbi Mouse homolog of the yeast origin recognition complex subunit ORC1 and chromosomal localization of the cognate mouse gene Orc1
    P Zisimopoulou
    Institute of Biochemistry, Biozentrum, University of Wurzburg, Germany
    Mol Gen Genet 260:295-9. 1998
    b>ORC1 encodes a subunit of the eukaryotic origin recognition complex in yeast, which has been shown to play a crucial role in chromosomal DNA replication and transcriptional silencing...
  96. ncbi Can hydridic-to-protonic hydrogen bonds catalyze hydride transfers in biological systems?
    Simona Marincean
    Natural Sciences Department, University of Michigan Dearborn, Dearborn, Michigan 48128 1491, United States
    J Phys Chem A 114:13376-80. 2010
    Catalysis of hydride transfer by hydridic-to-protonic hydrogen (HHH) bonding in α-hydroxy carbonyl isomerization reactions was examined computationally in the lithium salts of 7-substituted endo-3-hydroxybicyclo[2.2.1]hept-5-en-2-ones...
  97. ncbi Determination of urinary orotic acid and uracil by capillary zone electrophoresis
    C Salerno
    Institute of Paediatrics and Department of Biochemical Sciences, University of Roma La Sapienza, Rome, Italy
    J Chromatogr B Biomed Sci Appl 734:175-8. 1999
    ..2. The method was applied for studying a patient with HHH (hyperomithinemia, hyperammonemia and homocitrullinuria) syndrome...
  98. doi Function of the origin recognition complex 1 (ORC1) outside DNA replication in Drosophila
    So Young Park
    The Ohio State Comprehensive Cancer Center, The Ohio State University, Columbus, OH, USA
    Cell Cycle 10:3957-63. 2011
    ..Levels of the largest ORC subunit, ORC1, oscillate during the mitotic cell cycle and regulate origin usage...
  99. pmc Protection of repetitive DNA borders from self-induced meiotic instability
    Gerben Vader
    Whitehead Institute for Biomedical Research, 9 Cambridge Center, Cambridge, Massachusetts 02142, USA
    Nature 477:115-9. 2011
    ..border-specific protection system consisting of the meiotic ATPase Pch2 and the origin recognition complex subunit Orc1. Upon disruption of these factors, DSB formation and recombination increased specifically in the outermost rDNA ..
  100. pmc Single Qdot-labeled glycosylase molecules use a wedge amino acid to probe for lesions while scanning along DNA
    Andrew R Dunn
    The Department of Microbiology and Molecular Genetics, University of Vermont, Burlington, VT 05405, USA
    Nucleic Acids Res 39:7487-98. 2011
    ..DNA glycosylase (Fpg) and endonuclease VIII (Nei) are members of two glycosylase families: the helix-hairpin-helix (HhH) superfamily and the Fpg/Nei family...
  101. doi Different roles of the human Orc6 protein in the replication initiation process
    Andreas W Thomae
    Department of Gene Vectors, Helmholtz Zentrum Munchen, German Research Center for Environmental Health, Munich, Germany
    Cell Mol Life Sci 68:3741-56. 2011
    ..We show that Orc6 not only interacts with Orc1-Orc5 but also with the initiation factor Cdc6...

Research Grants66

  1. Molecular Basis of OTC Deficiency and Related Disorders
    Mendel Tuchman; Fiscal Year: 2005
    ..properties of human native and recombinant wild type mitochondrial ornithine carrier (ORNT) and mutants causing HHH syndrome (a) Study the oligomeric structure of ORNT by using hydrodynamic analysis, gel filtration, charge shift ..
  2. Structure/Function Studies of DNA Replication Initiation
    James Berger; Fiscal Year: 2007
    ..Specifically, we aim to: 1) biochemically and structurally determine how the archaeal Cdc6/Orc1 protein interacts with specific replication origin sites, 2) reconstitute and biophysically characterize dimeric ..
  3. New Perspectives in Transporter Biology (FASEB Conference)
    Michael Robinson; Fiscal Year: 2005
    ..attention deficit hyperactivity disorder (ADHD), diabetes, orthostatic hypotension, cystinuria, cystic fibrosis, HHH syndrome, etc; there is a growing list of 'transporteropathies'...
  4. Optimization of hybrid enzymes for DNA technologies
    Alexei Slesarev; Fiscal Year: 2006
    ..Fidelity Systems has successfully pursued this strategy by recognizing the role of helixhairpin- helix (HhH) protein domains in sequence non-specific interactions with DNA...
  5. DNA POLYMERASE BETA AND MUTAGENESIS
    Joann B Sweasy; Fiscal Year: 2010
    ..synthesis are located within regions of Pol ss other than the hydrophobic hinge, loop II, and the region between HhH motifs and to study mechanisms used by specific regions of Pol ss that are critical for accurate DNA synthesis...
  6. CELL CYCLE OF PLASMODIUM FALCIPARUM
    Debopam Chakrabarti; Fiscal Year: 2003
    ..falciparum homologues of MCM4, ORC1 and ORC5. (b) Identify proteins interacting with P...
  7. SLEEP HEART HEALTH STUDY
    Helaine Resnick; Fiscal Year: 2007
    ..Accomplishing these goals will provide important information concerning the public health impact of SDB. ..
  8. DNA replication checkpoints in fission yeast
    GENNARO D URSO; Fiscal Year: 2007
    ..Moreover, our results will likely identify new drug targets for cancer therapy, and reveal potentially less mutagenic strategies to block cell cycle progression in cancer cells. ..
  9. Alzheimer Caregiver Coping: Mental and Physical Health
    Igor Grant; Fiscal Year: 2007
    ..This study will provide a basis for a mechanistic understanding of the path from CG stress to CV morbidity in elderly CG, and opens the possibility of targeted interventions to reduce this risk. ..
  10. California NeuroAIDS Tissue Network
    Igor Grant; Fiscal Year: 2007
    ..The CNTN will coordinate its work closely with that of the other NNTC sites to assure that the entire NNTC develops as a national and international resource for research on neuroAIDS. ..
  11. Alzheimer Caregiver Coping: Mental and Physical Health
    Igor Grant; Fiscal Year: 2010
    ..This study will provide a basis for a mechanistic understanding of the path from CG stress to CV morbidity in elderly CG, and opens the possibility of targeted interventions to reduce this risk. ..
  12. INTERNATIONAL CORE
    Igor Grant; Fiscal Year: 2007
    ..abstract_text> ..
  13. Role of Arginase in Inflammation and Trauma
    SIDNEY MACHEN MORRIS; Fiscal Year: 2010
    ..Results of these studies will provide a basis for rational development of new and more effective therapeutic strategies and for monitoring efficacy of treatment in inflammatory diseases. ..
  14. Role of Arginase in Inflammation and Trauma
    SIDNEY MACHEN MORRIS; Fiscal Year: 2011
    ..Results of these studies will provide a basis for rational development of new and more effective therapeutic strategies and for monitoring efficacy of treatment in inflammatory diseases. ..
  15. ARGINASE AND INFLAMMATION AND TRAUMA
    Sidney Morris; Fiscal Year: 2001
    ..The third aim is to elucidate the physiologic roles of Arg II in vivo. The investigator will apply tools of modern molecular biology and genetics to address these questions. ..
  16. NEW INHIBITORS FOR THE PENICILLIN BINDING PROTEINS
    WILLIAM GUTHEIL; Fiscal Year: 2002
    ..These studies will established improved methods for the study of the PBPs, determine essential features of PBP substrate specificity, and use this information to design new PBP inhibitors. ..
  17. Cell cycle control by ORC1 and other E2F targets
    Maki Asano; Fiscal Year: 2005
    ..Three specific aims will be pursued. (1) Identification of the mechanisms underlying ORC1 degradation...