Genomes and Genes
Gene Symbol: SLC25A15
Description: solute carrier family 25 member 15
Alias: D13S327, HHH, ORC1, ORNT1, mitochondrial ornithine transporter 1, ornithine transporter 1, solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15
Publications180 found, 100 shown here
- Insights into the mutation-induced HHH syndrome from modeling human mitochondrial ornithine transporter-1Jing 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...
- Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporterJ 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 ..
- Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndromeS 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 ..
- Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndromeS 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.
- Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndromeS 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)...
- A novel mutation, P126R, in a Japanese patient with HHH syndromeTakeshi 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...
- The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoformsGiuseppe 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. 2003Two 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...
- Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional studyAlessandra 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...
- Essential and non-essential DNA replication genes in the model halophilic Archaeon, Halobacterium sp. NRC-1Brian 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...
- 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 disorderJosé 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. 2003We recently characterized the mitochondrial ornithine transporter (ORNT1), the gene defective in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, a urea cycle disorder...
- Crystal structures of human DNA polymerase beta complexed with DNA: implications for catalytic mechanism, processivity, and fidelityH 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 ..
- A genetic screen for replication initiation defective (rid) mutants in Schizosaccharomyces pombeAlexandra 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...
- Genomewide and biochemical analyses of DNA-binding activity of Cdc6/Orc1 and Mcm proteins in Pyrococcus spFujihiko 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 ..
- Genetic and physical mapping of DNA replication origins in Haloferax volcaniiCedric 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 ..
- Genetic variation in the urea cycle: a model resource for investigating key candidate genes for common diseasesSabrina 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 ..
- Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failureA 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)...
- Crystal structure of an archaeal Ski2p-like protein from Pyrococcus horikoshii OT3Xiaodong 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...
- Novel adhesin from Pasteurella multocida that binds to the integrin-binding fibronectin FnIII9-10 repeatsLisa 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 ..
- Functional changes in a novel uracil-DNA glycosylase determined by mutational analysesE 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 ..
- Octahedral tilting in Pb-based relaxor ferroelectrics at high pressureBernd J Maier
Department Geowissenschaften, Universitat Hamburg, 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...
- Intra-arterial verapamil-induced seizures: case report and review of the literatureFranklin 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...
- Structure and activity of a thermostable thymine-DNA glycosylase: evidence for base twisting to remove mismatched normal DNA basesClifford 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...
- Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndromeJosé 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...
- Orotic acid quantification in dried blood spots and biological fluids by hydrophilic interaction liquid chromatography tandem mass spectrometryOceania 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...
- The HhH2/NDD domain of the Drosophila Nod chromokinesin-like protein is required for binding to chromosomes in the oocyte nucleusWei 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...
- Trypanosome prereplication machinery contains a single functional orc1/cdc6 protein, which is typical of archaeaPatrícia 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/..
- HHH regime for arteritis secondary to TB meningitis: a prospective randomized studyArunodaya 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...
- Mutational studies of Pa-AGOG DNA glycosylase from the hyperthermophilic crenarchaeon Pyrobaculum aerophilumGondichatnahalli 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...
- The cyclin A centrosomal localization sequence recruits MCM5 and Orc1 to regulate centrosome reduplicationRebecca 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...
- Safety of intrathecal sodium nitroprusside for the treatment and prevention of refractory cerebral vasospasm and ischemia in humansJ 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...
- Is hereditary hemochromatosis a balanced polymorphism: an analysis of family size among hemochromatosis heterozygotesR 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...
- Health consequences of Alzheimer's caregiving transitions: effects of placement and bereavementIgor Grant
Department of Psychiatry, University of California, San Diego, La Jolla, California 92093 0680, USA
Psychosom Med 64:477-86. 2002....
- Binding of AlF-C, an Orc1-binding transcriptional regulator, enhances replicator activity of the rat aldolase B originHiroyuki 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...
- Cdc6/Orc1 from Pyrococcus furiosus may act as the origin recognition protein and Mcm helicase recruiterMasaki 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. 2010Archaea have one or more Cdc6/Orc1 proteins, which share sequence similarities with eukaryotic Cdc6 and Orc1...
- The archaeal cell cycle: current issuesRolf Bernander
Department of Molecular Evolution, Evolutionary Biology Center, Uppsala University, Norbyvagen 18C, SE 752 36 Uppsala, Sweden
Mol Microbiol 48:599-604. 2003The recently discovered structural similarities between the archaeal Orc1/Cdc6 and bacterial DnaA initiator proteins for chromosome replication have exciting implications for cell cycle regulation...
- Localized melting of duplex DNA by Cdc6/Orc1 at the DNA replication origin in the hyperthermophilic archaeon Pyrococcus furiosusFujihiko 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...
- Pa-AGOG, the founding member of a new family of archaeal 8-oxoguanine DNA-glycosylasesAlessandro 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...
- Chromosome replication dynamics in the archaeon Sulfolobus acidocaldariusIain 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...
- Deoxyribose phosphate excision by the N-terminal domain of the polymerase beta: the mechanism revisitedJ 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...
- Hormonal induction of hepatic mitochondrial ornithine/citrulline transporter mRNASidney 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 ..
- Over-expression of human DNA polymerase lambda in E. coli and characterization of the recombinant enzymeNoriko 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...
- A DNA glycosylase from Pyrobaculum aerophilum with an 8-oxoguanine binding mode and a noncanonical helix-hairpin-helix structureGondichatnahalli 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...
- Proteomic analysis of human O6-methylguanine-DNA methyltransferase by affinity chromatography and tandem mass spectrometrySuryakant 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,..
- Elaboration, diversification and regulation of the Sir1 family of silencing proteins in SaccharomycesJennifer 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, ..
- Transcriptional silencing functions of the yeast protein Orc1/Sir3 subfunctionalized after gene duplicationMeleah 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, ..
- Characterization of the metal ion binding helix-hairpin-helix motifs in human DNA polymerase beta by X-ray structural analysisH 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...
- The essential role of Saccharomyces cerevisiae CDC6 nucleotide-binding site in cell growth, DNA synthesis, and Orc1 associationB 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...
- Conformational changes induced by nucleotide binding in Cdc6/ORC from Aeropyrum pernixMartin 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...
- 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 miceS 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 ..
- Selective instability of Orc1 protein accounts for the absence of functional origin recognition complexes during the M-G(1) transition in mammalsD 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...
- Novel splicing variant of mouse Orc1 is deficient in nuclear translocation and resistant for proteasome-mediated degradationYasuyuki 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...
- Functional differentiation and cooperative interaction between two eukaryote-like archaeal Orc1/Cdc6 proteins on the replication originPei 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. 2007The 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...
- Structure-guided mutational analysis of the OB, HhH, and BRCT domains of Escherichia coli DNA ligaseLi 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...
- Mammary sensitivity to protein restriction and re-alimentationM 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) ..
- Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesiaJ 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)...
- Cerebral Vasospasm Following Subarachnoid HemorrhageM Akif Topcuoglu
Neurovascular Surgery, Massachusetts General Hospital, 55 Fruit Street, VBK 802, Boston, MA 02114, USA
Curr Treat Options Cardiovasc Med 4:373-384. 2002..Elevated TCD velocities often initiate the use of triple-H (HHH: hypertension, hemodilution, and hypervolemia) therapy and subsequently guide it...
- Solution structure and DNA-binding properties of the C-terminal domain of UvrC from E.coliS Singh
Bijvoet Center for Biomolecular Research, Utrecht University, Padualaan 8, 3584 CH Utrecht, The Netherlands
EMBO J 21:6257-66. 2002..The structure shows two helix-hairpin-helix (HhH) motifs connected by a small connector helix. The UvrC CTD is shown to mediate structure-specific DNA binding...
- The 'ORC cycle': a novel pathway for regulating eukaryotic DNA replicationMelvin L DePamphilis
National Institute of Child Health and Human Development, Building 6 416, 9000 Rockville Pike, National Institutes of Health, Bethesda, MD 20892 2753, USA
Gene 310:1-15. 2003..In mammals, the affinity of Orc1 for chromatin is selectively reduced during S-phase and restored during early G1-phase...
- Crystal structures of 3-methyladenine DNA glycosylase MagIII and the recognition of alkylated basesBrandt F Eichman
Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, 240 Longwood Avenue, Boston, MA 02115, USA
EMBO J 22:4898-909. 2003..We report base excision and DNA binding activities of MagIII active site mutants, together with a structural comparison of the HhH glycosylases.
- Tuning the decay time of lanthanide-based near infrared luminescence from micro- to milliseconds through d-->f energy transfer in discrete heterobimetallic complexesStéphane Torelli
Department of Inorganic, Analytical and Applied Chemistry, University of Geneva, 30 quai E Ansermet, 1211 Geneva 4, Switzerland
Chemistry 11:3228-42. 2005..and Ru(II)N6 chromophores have been incorporated by self-assembly into heterobimetallic triple-stranded helicates HHH-[CrLnL3]6+ and HHH-[RuLnL3]5+...
- Biochemical characterization of two Cdc6/ORC1-like proteins from the crenarchaeon Sulfolobus solfataricusMariarita De Felice
Istituto di Biochimica delle Proteine, Consiglio Nazionale delle Ricerche, Via P Castellino, 111, 80131, Napoli, Italy
Extremophiles 10:61-70. 2006..to the eukaryal replication initiation factors of cell division control (Cdc6) and origin recognition complex (ORC1), has not yet been clearly established...
- A novel motif governs APC-dependent degradation of Drosophila ORC1 in vivoMarito Araki
Department of Molecular Genetics and Microbiology, Duke University Medical Center, Durham, North Carolina 27710, USA
Genes Dev 19:2458-65. 2005..In Drosophila, the largest subunit of the origin recognition complex protein 1 (ORC1) is degraded at the end of M phase and throughout much of G1 by anaphase-promoting complexes (APC) activated by Fzr/..
- Archaeal cell cycle progressMagnus Lundgren
Department of Molecular Evolution, Evolutionary Biology Center, Uppsala University, Norbyvagen 18C, SE 752 36 Uppsala, Sweden
Curr Opin Microbiol 8:662-8. 2005..DNA elements--origin recognition boxes--have been identified in the origins of replication, and shown to bind the Orc1/Cdc6 proteins involved in cell cycle control...
- A novel branching enzyme of the GH-57 family in the hyperthermophilic archaeon Thermococcus kodakaraensis KOD1Taira Murakami
Department of Synthetic Chemistry and Biological Chemistry, Graduate School of Engineering, Kyoto University, Katsura, Nishikyo ku, Kyoto 615 8510, Japan
J Bacteriol 188:5915-24. 2006..At the C terminus of the TK1436 protein, two copies of a helix-hairpin-helix (HhH) motif were found...
- Interaction between HP1alpha and replication proteins in mammalian cellsTanja Auth
Institute of Genetics, University of Bonn, 53117 Bonn, Germany
Exp Cell Res 312:3349-59. 2006..two-hybrid system and immunoprecipitation, we report here that murine HP1alpha interacts with the preRC proteins ORC1, ORC2 and CDC6...
- The CENP-B homolog, Abp1, interacts with the initiation protein Cdc23 (MCM10) and is required for efficient DNA replication in fission yeastAlexandra M Locovei
University of Miami School of Medicine, Department of Molecular and Cellular Pharmacology, Miami, FL 33101, USA
Cell Div 1:27. 2006..interactions were also observed between abp1+ and four additional DNA replication initiation genes cdc18+, cdc21+, orc1+, and orc2+...
- Home telemonitoring of vital signs and cardiorespiratory signals in heart failure patients: system architecture and feasibility of the HHH modelGian Domenico Pinna
Department of Biomedical Engineering, Salvatore Maugeri Foundation IRCCS, Scientific Institute of Montescano, Montescano PV, Italy
Int J Cardiol 120:371-9. 2007The Home or Hospital in Heart Failure Study (HHH) is a European Community funded trial (QLGA-CT-2001-02424) which compares usual care of heart failure (HF) with three home-based interventions in a multicenter, multicountry (Italy, Poland ..
- Cyclin and cyclin-dependent kinase substrate requirements for preventing rereplication reveal the need for concomitant activation and inhibitionAmy E Ikui
The Rockefeller University, New York, New York 10021, USA
Genetics 175:1011-22. 2007..by cyclin-Cdk-dependent phosphorylation of the prereplicative complex (pre-RC) components Mcm2-7, Cdc6, and Orc1-6. Several of these controls must be simultaneously blocked by mutation to obtain rereplication...
- [Expression of origin recognition complex 1 gene in DNA replication of vascularsmooth muscle cells]Ming Hong Jiang
Department of Cardiology, South West Hospital, Third Military Medical University, Chongqing 400038, China
Zhonghua Bing Li Xue Za Zhi 36:122-5. 2007To explore the expression of origin recognition complex 1 (ORC1) during the DNA replication of vascular muscle cells (VSMC).
- Thermodynamic parameters governing the self-assembly of head-head-head lanthanide bimetallic helicatesThomas B Jensen
Laboratory of Lanthanide Supramolecular Chemistry, Ecole Polytechnique Federale de Lausanne, LCSL BCH 1401 Lausanne, Switzerland
Chemistry 13:8404-10. 2007..The percentage of HHH (head-head-head) isomer, in which each of the three ligand strands coordinates to the same lanthanide ion with the ..
- Divergent functions of multiple eukaryote-like Orc1/Cdc6 proteins on modulating the loading of the MCM helicase onto the origins of the hyperthermophilic archaeon Sulfolobus solfataricus P2Pei Xia Jiang
National Key Laboratory of Agricultural Microbiology, Center for Proteomics Research, College of Life Science and Technology, Huazhong Agricultural University, Wuhan 430070, China
Biochem Biophys Res Commun 361:651-8. 2007..In the current work, three eukaryotic Orc1/Cdc6 homologs (SsoCdc6-1, -2, and -3) from crenarchaeon Sulfolobus solfataricus were shown to have totally ..
- Incorporating biochemical information and backbone flexibility in RosettaDock for CAPRI rounds 6-12Sidhartha Chaudhury
Program in Molecular and Computational Biophysics, Johns Hopkins University, Baltimore, Maryland 21218, USA
Proteins 69:793-800. 2007..In the case of Target 21, Orc1/Sir1, this resulted in a successful docking prediction where RosettaDock alone or with simple site constraints ..
- Monitoring response to treatment in melanoma patients: potential of a serum glycomic markerSenthamil R Selvan
Cell Biology Laboratory, Hoag Cancer Center, Newport Beach, CA 92663, USA
Int J Cancer 122:1374-83. 2008..24, patients were categorized into cohorts-I (LLL, n = 16), II (HHL/HLL, n = 4), III (LLH/LHH/LHL, n = 7) and IV (HHH/HLH, n = 7). The cohorts were regrouped as sTG- downregulators (sTG-DR; n = 20) and upregulators (sTG-UR; n = 14)...
- APC/CFzr/Cdh1 promotes cell cycle progression during the Drosophila endocycleKarine Narbonne-Reveau
Cell Biology and Metabolism Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Development 135:1451-61. 2008..but is cyclic, as demonstrated by the APC/C-dependent oscillation of the pre-replication complex component Orc1. Taken together, our data suggest a model in which the cyclic activity of APC/C Fzr/Cdh1 during the Drosophila ..
- RNA interference targeting ORC1 gene suppresses the proliferation of vascular smooth muscle cells in ratsMao 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...
- [Protective effect of hypotensive fluid resuscitation against lung ischemia-reperfusion injury in pregnant rabbits with uncontrolled hemorrhagic shock]Wei Qin
Department of Obstetrics and Gynecology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China
Nan Fang Yi Ke Da Xue Xue Bao 28:1042-4. 2008..To evaluate the effects of different fluid resuscitation strategies on superoxide dismutase (SOD), malondialdehyde (MDA) and myeloperoxidase (MPO) activities in the lung tissue in pregnant rabbits with uncontrolled hemorrhagic shock...
- Involvement of human ORC and TRF2 in pre-replication complex assembly at telomeresYasutoshi Tatsumi
Virology Division, National Cancer Center Research Institute, 5 1 1 Tsukiji, Chuohku, Tokyo 104 0045, Japan
Genes Cells 13:1045-59. 2008..a telomere sequence-binding protein that protects telomeres and functions in telomere length homeostasis, via the ORC1 subunit...
- Orc1 controls centriole and centrosome copy number in human cellsAdriana S Hemerly
Cold Spring Harbor Laboratory, 1 Bungtown Road, Cold Spring Harbor 11724, NY, USA
Science 323:789-93. 2009..We report a new role for the Orc1 protein, a subunit of the origin recognition complex (ORC) that is a key component of the DNA replication licensing ..
- The human and mouse SLC25A29 mitochondrial transporters rescue the deficient ornithine metabolism in fibroblasts of patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndromeJosé 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)...
- DNA replication and the GINS complex: localization on extended chromatin fibersStephanie M Cohen
Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA
Epigenetics Chromatin 2:6. 2009..Here, we show the organization of GINS complexes on extended chromatin fibers in relation to sites of DNA replication and replication-associated proteins...
- Use of health information technology in home health and hospice agencies: United States, 2007Helaine E Resnick
American Association of Homes and Services for the Aging, Washington, DC, USA
J Am Med Inform Assoc 17:389-95. 2010This report provides updated estimates on use of electronic medical records (EMRs) in US home health and hospice (HHH) agencies, describes utilization of EMR functionalities, and presents novel data on telemedicine and point of care ..
- Trypanosoma cruzi DNA replication includes the sequential recruitment of pre-replication and replication machineries close to nuclear peripherySimone Guedes Calderano
Laboratorio de Parasitologia, Instituto Butantan, Universidade Federal de Sao Paulo, Sao Paulo, Brazil
Nucleus 2:136-45. 2011..We followed Orc1/Cdc6, a pre-replication machinery component and the proliferating cell nuclear antigen (PCNA), a component of ..
- Identification of one of the apurinic/apyrimidinic lyase active sites of topoisomerase V by structural and functional studiesRakhi Rajan
Department of Molecular Biosciences, Northwestern University, 2205 Tech Drive, Evanston, IL 60208, USA
Nucleic Acids Res 41:657-66. 2013..the topoisomerase domain spans the N-terminus of the protein and is followed by 12 tandem helix-hairpin-helix [(HhH)(2)] domains...
- The role of PR-Set7 in replication licensing depends on Suv4-20hDavid B Beck
Howard Hughes Medical Institute, Department of Biochemistry, New York University School of Medicine, New York, NY 10016, USA
Genes Dev 26:2580-9. 2012..with this, H4K20 methylation status plays a direct role in recruiting ORC through the binding properties of ORC1 and ORCA/LRWD1...
- Genome-wide mapping of human DNA-replication origins: levels of transcription at ORC1 sites regulate origin selection and replication timingGaetano Ivan Dellino
Department of Experimental Oncology, European Institute of Oncology, 20141 Milan, Italy
Genome Res 23:1-11. 2013We report the genome-wide mapping of ORC1 binding sites in mammals, by chromatin immunoprecipitation and parallel sequencing (ChIP-seq)...
- Helping Her Heal-Group: a pilot study to evaluate a group delivered educational intervention for male spouses of women with breast cancerJennifer M Jones
Cancer Survivorship Program, Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada
Psychooncology 22:2102-9. 2013..In response, we developed a five-session group educational counselling intervention (Helping Her Heal-Group (HHH-G)) for husbands of women with early-stage breast cancer...
- The level of origin firing inversely affects the rate of replication fork progressionYuan Zhong
Molecular and Computational Biology Program, University of Southern California, Los Angeles, CA 90089, USA
J Cell Biol 201:373-83. 2013..Depletion of Orc1, which acts in origin firing differently than Cdc7, had similar effects as Cdc7 depletion, consistent with ..
- Human origin recognition complex binds preferentially to G-quadruplex-preferable RNA and single-stranded DNAShoko Hoshina
From the Department of Chemical and Biological Sciences, Faculty of Science, Japan Women s University, Tokyo 112 8681, Japan
J Biol Chem 288:30161-71. 2013Origin recognition complex (ORC), consisting of six subunits ORC1-6, is known to bind to replication origins and function in the initiation of DNA replication in eukaryotic cells...
- Ab initio coordination chemistry for nickel chelation motifsR Jesu Jaya Sudan
Bioinformatics Division, SBST, VIT University, Vellore, 632 014, India
PLoS ONE 10:e0126787. 2015..The most common chelates identified were histidine based namely HHH, HDH, HEH and HH spaced at specific intervals...
- Regulation of Plasmodium falciparum Origin Recognition Complex subunit 1 (PfORC1) function through phosphorylation mediated by CDK-like kinase PK5Abhijit S Deshmukh
Special Centre for Molecular Medicine, Jawaharlal Nehru University, New Delhi, India
Mol Microbiol 98:17-33. 2015..Upon disassembly of telomeric clusters at later stages of parasite development, ORC1 is distributed in the nucleus and parasite cytoplasm where it may be required for its other cellular functions ..
- De Novo GMNN Mutations Cause Autosomal-Dominant Primordial Dwarfism Associated with Meier-Gorlin SyndromeLindsay C Burrage
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA Department of Pediatrics, Texas Children s Hospital, Houston, TX 77030, USA
Am J Hum Genet 97:904-13. 2015..be caused by biallelic loss-of-function mutations in one of five genes encoding pre-replication complex proteins: ORC1, ORC4, ORC6, CDT1, and CDC6. Mutations in these genes cause disruption of the origin of DNA replication initiation...
- Adenine Glycosylase MutY of Corynebacterium pseudotuberculosis presents the antimutator phenotype and evidences of glycosylase/AP lyase activity in vitroRafael Cançado de Faria
Laboratory of Molecular Biology, Federal University of Sao Joao del Rei CCO, Av Sebastião Gonçalves Coelho, 400, Divinópolis, MG 35501 296, Brazil Electronic address
Infect Genet Evol 44:318-29. 2016..as catalytic residues involved in glycosylase/AP lyase activity and structural DNA-binding elements, such as the HhH motif and the [4Fe-4S] cluster...
- Genome based identification and analysis of the pre-replicative complex of Arabidopsis thalianaH P Masuda
Departamento de Bioquimica Medica, ICB, Universidade Federal do Rio de Janeiro, 21941 590 Rio de Janeiro, RJ, Brazil
FEBS Lett 574:192-202. 2004..Different from the other eukaryotes, Arabidopsis houses in its genome two putative homologs of ORC1, CDC6 and CDT1...
- Studies of the properties of human origin recognition complex and its Walker A motif mutantsJennifer Giordano-Coltart
Molecular Biology Program, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, Box 97, New York, NY 10021, USA
Proc Natl Acad Sci U S A 102:69-74. 2005..the isolation of the wild-type Homo sapiens (Hs)ORC and variants containing a Walker A motif mutation in the Orc1, Orc4, or Orc5 subunit using the baculovirus-expression system...
- Overexpression of ORC subunits and increased ORC-chromatin association in transformed mammalian cellsAdrian J McNairn
Department of Biochemistry and Molecular Biology, S U N Y Upstate Medical University, Syracuse, New York 13210, USA
J Cell Biochem 96:879-87. 2005..ORC subunits 2 and 4 were highly overexpressed in both HeLa and VA13, whereas ORC1 levels were elevated in VA13 but considerably higher in HeLa cells...
- FAD24, a regulator of adipogenesis, is required for the regulation of DNA replication in cell proliferationYoshikazu Johmura
Department of Molecular Biology, Graduate School of Pharmaceutical Sciences, Nagoya City University, Nagoya, Aichi, Japan
Biol Pharm Bull 31:1092-5. 2008..that the knockdown inhibited DNA synthesis by preventing the recruitment of histone acetyltransferase binding to ORC1 (HBO1), a component of the pre-replicative complex, to origins...
- Assembly of the human origin recognition complex occurs through independent nuclear localization of its componentsSoma Ghosh
NICHD, National Institutes of Health, Bethesda, Maryland 20892 2753, USA
J Biol Chem 286:23831-41. 2011..Here we show that stable ORC(1-6) complexes also can be purified from human cell extracts and that Orc6 and Orc1 each contain a single nuclear localization signal that is essential for nuclear localization but not for ORC ..
- Asymmetric dimethylarginine is transported by the mitochondrial carrier SLC25A2Vito Porcelli
Laboratory of Biochemistry and Molecular Biology, Department of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari, Via Orabona 4, 70125, Bari, Italy
Amino Acids 48:427-36. 2016..and ornithine and in contrast to the other known mitochondrial amino acid transporters SLC25A12, SLC25A13, SLC25A15, SLC25A18, SLC25A22, and SLC25A29...
- ORC proteins in the mammalian zygoteMichael A Ortega
Institute for Biogenesis Research, Department of Anatomy, Biochemistry and Physiology, John A Burns School of Medicine, University of Hawaii at Manoa, 1960 East West Road, Honolulu, HI 96822, USA
Cell Tissue Res 363:195-200. 2016The origin recognition complex (ORC) proteins, ORC1-6, are the first known proteins that bind DNA replication origins to mark the competency for the initiation of DNA synthesis...
- Improving long term outcomes in urea cycle disorders-report from the Urea Cycle Disorders ConsortiumSusan E Waisbren
Boston Children s Hospital, Harvard Medical School, Boston, MA, USA
J Inherit Metab Dis 39:573-84. 2016..ARGD, Argininemia); Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome (or mitochondrial ornithine transporter 1 deficiency [ORNT1D]); and Citrullinemia type II (mitochondrial aspartate/glutamate carrier ..
- Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in miceLaila 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 (..
- Mendel Tuchman; Fiscal Year: 2016..aciduria);Arginase (ARG) deficiency (Argininemia);Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome;and Citrullinemia type II...
- Susan S Wallace; Fiscal Year: 2014..goals of this Program Project are to understand at the atomic level how three families of DNA repair enzymes the HhH-GPD superfamily of DNA glycosylases, the Fpg/Nei family of DNA glycosylases and the RecA-RAD51 family of ..
- Joann B Sweasy; Fiscal Year: 2016..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...
- 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'...
- Optimization of hybrid enzymes for DNA technologiesAlexei 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...
- CELL CYCLE OF PLASMODIUM FALCIPARUMDebopam Chakrabarti; Fiscal Year: 2003..falciparum homologues of MCM4, ORC1 and ORC5. (b) Identify proteins interacting with P...
- INTERNET-BASED HEALTHCARE SERVICES FOR THE HOMELESSDavid Wheeler; Fiscal Year: 2001DESCRIPTION (adapted from the Abstract): Healthcare for the Homeless-Houston (HHH) is a strategic alliance of health care providers and community-based agencies committed to improving the health of area homeless men, women, and children...
- SLEEP HEART HEALTH STUDYHelaine Resnick; Fiscal Year: 2007..Accomplishing these goals will provide important information concerning the public health impact of SDB. ..
- Regulation of Arginase Genes in Sepsis and TraumaSidney Morris; Fiscal Year: 2005..abstract_text> ..
- NEW INHIBITORS FOR THE PENICILLIN BINDING PROTEINSWILLIAM 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. ..
- Cell cycle control by ORC1 and other E2F targetsMaki Asano; Fiscal Year: 2005..Three specific aims will be pursued. (1) Identification of the mechanisms underlying ORC1 degradation...
- California NeuroAIDS Tissue NetworkIgor 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. ..
- DNA replication checkpoints in fission yeastGENNARO 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. ..