FANCA

Summary

Gene Symbol: FANCA
Description: Fanconi anemia complementation group A
Alias: FA-H, FA1, FAA, FACA, FAH, FANCH, Fanconi anemia group A protein, Fanconi anemia, complementation group H, Fanconi anemia, type 1
Species: human

Top Publications

  1. ncbi The dorsomedial hypothalamic nucleus is not necessary for the expression of circadian food-anticipatory activity in rats
    Glenn J Landry
    Department of Psychology, Simon Fraser University, Burnaby, BC, Canada
    J Biol Rhythms 22:467-78. 2007
  2. doi The role of free amino acids in semen of rainbow trout Oncorhynchus mykiss and carp Cyprinus carpio
    F Lahnsteiner
    Department for Organismic Biology, University of Salzburg, Hellbrunnerstrasse 34, 5020 Salzburg, Austria
    J Fish Biol 75:816-33. 2009
  3. ncbi Fanconi anemia protein complex: mapping protein interactions in the yeast 2- and 3-hybrid systems
    Susan M Gordon
    Program in Genetics and Genomic Biology, Research Institute, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
    Blood 102:136-41. 2003
  4. ncbi Expression cloning of a cDNA for the major Fanconi anaemia gene, FAA
    J R Lo ten Foe
    Department of Human Genetics, Free University, Amsterdam, The Netherlands
    Nat Genet 14:320-3. 1996
  5. ncbi Fanconi anemia proteins localize to chromatin and the nuclear matrix in a DNA damage- and cell cycle-regulated manner
    F Qiao
    Departments of Microbiology and Pediatrics, University of Virginia and the University of Virginia Health System, Charlottesville, Virginia 22908, USA
    J Biol Chem 276:23391-6. 2001
  6. ncbi Fanconi anemia protein, FANCA, associates with BRG1, a component of the human SWI/SNF complex
    T Otsuki
    Department of Hematology, Jichi Medical School, Yakushiji 3311 1, Minamikawachi, Kawachi, Tochigi 329 0498, Japan
    Hum Mol Genet 10:2651-60. 2001
  7. ncbi Phenotypic correction of primary Fanconi anemia T cells with retroviral vectors as a diagnostic tool
    Helmut Hanenberg
    Klinik für Pädiatrische Hämatologie und Onkologie, Zentrum fur Kinderheilkunde, Heinrich Heine Universitat, Dusseldorf, Germany
    Exp Hematol 30:410-20. 2002
  8. ncbi Heterogeneous activation of the Fanconi anemia pathway by patient-derived FANCA mutants
    Daiki Adachi
    Division of Genetic Diagnosis, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
    Hum Mol Genet 11:3125-34. 2002
  9. pmc A multiprotein nuclear complex connects Fanconi anemia and Bloom syndrome
    Amom Ruhikanta Meetei
    Laboratory of Genetics Mass Spectrometry Unit, National Institute on Aging NIH, TRIAD Center Room 3000, 333 Cassell Drive, Baltimore, MD 21224, USA
    Mol Cell Biol 23:3417-26. 2003
  10. ncbi A novel ubiquitin ligase is deficient in Fanconi anemia
    Amom Ruhikanta Meetei
    Laboratory of Genetics, National Institute on Aging, National Institutes of Health, 333 Cassell Drive, TRIAD Center Room 3000, Baltimore, Maryland 21224, USA
    Nat Genet 35:165-70. 2003

Research Grants

  1. Mice Humanized with Hepatocytes and iPS Cells from Patients with Metabolic Diseas
    Stephen C Strom; Fiscal Year: 2010
  2. FANCD2 Monoubiquitination in DNA Damage Responses
    Paul R Andreassen; Fiscal Year: 2012
  3. Qishen Pang; Fiscal Year: 2015
  4. Lentiviral Vectors for the Treatment of Fanconi Anemia
    DAVID W CLAPP; Fiscal Year: 2010
  5. Double Humanization of Liver and Blood in Mice
    ELIZABETH MARIE WILSON; Fiscal Year: 2012
  6. FAH-Deficient Pigs
    Scott L Nyberg; Fiscal Year: 2011
  7. FAA AND FAD GENES AND PROTEINS
    Manuel Buchwald; Fiscal Year: 1999
  8. Markus Grompe; Fiscal Year: 2016
  9. FANCONI ANEMIA--PATHOPHYSIOLOGY AND TREATMENT
    Christopher Walsh; Fiscal Year: 1999
  10. YPE PETER DE JONG; Fiscal Year: 2015

Detail Information

Publications333 found, 100 shown here

  1. ncbi The dorsomedial hypothalamic nucleus is not necessary for the expression of circadian food-anticipatory activity in rats
    Glenn J Landry
    Department of Psychology, Simon Fraser University, Burnaby, BC, Canada
    J Biol Rhythms 22:467-78. 2007
    Restricted daytime feeding generates food-anticipatory activity (FAA) by entrainment of a circadian pacemaker separate from the light-entrainable pacemaker located in the SCN...
  2. doi The role of free amino acids in semen of rainbow trout Oncorhynchus mykiss and carp Cyprinus carpio
    F Lahnsteiner
    Department for Organismic Biology, University of Salzburg, Hellbrunnerstrasse 34, 5020 Salzburg, Austria
    J Fish Biol 75:816-33. 2009
    The present study investigated (1) the free amino acid (FAA) composition in semen of rainbow trout Oncorhynchus mykiss and carp Cyprinus carpio, (2) enzyme systems involved in amino acid metabolism and (3) the effect of amino acids on ..
  3. ncbi Fanconi anemia protein complex: mapping protein interactions in the yeast 2- and 3-hybrid systems
    Susan M Gordon
    Program in Genetics and Genomic Biology, Research Institute, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
    Blood 102:136-41. 2003
    ..Eight FA complementation groups have been identified. The FANCA, FANCC, FANCE, FANCF, and FANCG proteins form a nuclear complex required for the monoubiquination of the FANCD2 ..
  4. ncbi Expression cloning of a cDNA for the major Fanconi anaemia gene, FAA
    J R Lo ten Foe
    Department of Human Genetics, Free University, Amsterdam, The Netherlands
    Nat Genet 14:320-3. 1996
    ..3 (ref.8). Genetic map positions were recently reported for two more FA genes, FAA (16q24.3) and FAD (3p22-26)...
  5. ncbi Fanconi anemia proteins localize to chromatin and the nuclear matrix in a DNA damage- and cell cycle-regulated manner
    F Qiao
    Departments of Microbiology and Pediatrics, University of Virginia and the University of Virginia Health System, Charlottesville, Virginia 22908, USA
    J Biol Chem 276:23391-6. 2001
    ..The FancA (Fanconi anemia complementation group A), FancC, and FancG proteins have been detected within a nuclear complex, ..
  6. ncbi Fanconi anemia protein, FANCA, associates with BRG1, a component of the human SWI/SNF complex
    T Otsuki
    Department of Hematology, Jichi Medical School, Yakushiji 3311 1, Minamikawachi, Kawachi, Tochigi 329 0498, Japan
    Hum Mol Genet 10:2651-60. 2001
    ..We identified an interaction between the FA protein, FANCA and brm-related gene 1 (BRG1) product...
  7. ncbi Phenotypic correction of primary Fanconi anemia T cells with retroviral vectors as a diagnostic tool
    Helmut Hanenberg
    Klinik für Pädiatrische Hämatologie und Onkologie, Zentrum fur Kinderheilkunde, Heinrich Heine Universitat, Dusseldorf, Germany
    Exp Hematol 30:410-20. 2002
    ..The aim of this study was to develop a rapid laboratory procedure that is capable of subtyping Fanconi anemia (FA) complementation groups FA-A, FA-C, FA-G, and FA-nonACG patients from a small amount of peripheral blood...
  8. ncbi Heterogeneous activation of the Fanconi anemia pathway by patient-derived FANCA mutants
    Daiki Adachi
    Division of Genetic Diagnosis, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
    Hum Mol Genet 11:3125-34. 2002
    ..is growing evidence for a model of the FA pathway, wherein a nuclear multiprotein complex of five FA proteins (FANCA, C, E, F and G) regulates activation of FANCD2 into a monoubiquitinated form, which, collaborating with the BRCA1 ..
  9. pmc A multiprotein nuclear complex connects Fanconi anemia and Bloom syndrome
    Amom Ruhikanta Meetei
    Laboratory of Genetics Mass Spectrometry Unit, National Institute on Aging NIH, TRIAD Center Room 3000, 333 Cassell Drive, Baltimore, MD 21224, USA
    Mol Cell Biol 23:3417-26. 2003
    ..The findings that FA proteins are part of a DNA-unwinding complex imply that FA proteins may participate in DNA repair...
  10. ncbi A novel ubiquitin ligase is deficient in Fanconi anemia
    Amom Ruhikanta Meetei
    Laboratory of Genetics, National Institute on Aging, National Institutes of Health, 333 Cassell Drive, TRIAD Center Room 3000, Baltimore, Maryland 21224, USA
    Nat Genet 35:165-70. 2003
    ..Our data suggest that PHF9 has a crucial role in the Fanconi anemia pathway as the likely catalytic subunit required for monoubiquitination of FANCD2...
  11. pmc A human ortholog of archaeal DNA repair protein Hef is defective in Fanconi anemia complementation group M
    Amom Ruhikanta Meetei
    Division of Experimental Hematology, Cincinnati Children s Hospital Research Foundation and University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, Ohio 45229, USA
    Nat Genet 37:958-63. 2005
    ..Our data suggest an evolutionary link between Fanconi anemia-associated proteins and DNA repair; FANCM may act as an engine that translocates the Fanconi anemia core complex along DNA...
  12. ncbi Identification of FAAP24, a Fanconi anemia core complex protein that interacts with FANCM
    Alberto Ciccia
    Cancer Research UK, London Research Institute, Clare Hall Laboratories, South Mimms, Herts, UK
    Mol Cell 25:331-43. 2007
    ..Our data indicate that the FANCM/FAAP24 complex may play a key role in recruitment of the FA core complex to damaged DNA...
  13. pmc Cell cycle-dependent chromatin loading of the Fanconi anemia core complex by FANCM/FAAP24
    Jung Min Kim
    Department of Radiation Oncology, Dana Farber Cancer Institute, Harvard Medical School, Boston, MA, USA
    Blood 111:5215-22. 2008
    ..Dysregulated loading of the FA core complex accounts, at least in part, for the characteristic cellular and developmental abnormalities in FA...
  14. pmc RAD18-mediated ubiquitination of PCNA activates the Fanconi anemia DNA repair network
    Liyi Geng
    Division of Oncology Research, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    J Cell Biol 191:249-57. 2010
    ..Collectively, these experiments identify RAD18-mediated PCNA monoubiquitination as a central hub for the mobilization of the FA pathway by promoting FANCL-mediated FANCD2 monoubiquitylation...
  15. pmc Fanconi anemia complementation group A (FANCA) protein has intrinsic affinity for nucleic acids with preference for single-stranded forms
    Fenghua Yuan
    Department of Biochemistry and Molecular Biology, University of Miami Miller School of Medicine, Miami, Florida 33136, USA
    J Biol Chem 287:4800-7. 2012
    The Fanconi anemia complementation group A (FANCA) gene is one of 15 disease-causing genes and has been found to be mutated in ∼60% of Fanconi anemia patients...
  16. ncbi Novel mutations of the FANCG gene causing alternative splicing in Japanese Fanconi anemia
    T Yamada
    Radiation Biology Center, Kyoto University, Japan
    J Hum Genet 45:159-66. 2000
    ..Previously, we studied mutations of the FANCA gene, responsible for FA-A, and found pathogenic mutations in 12 of 15 unclassified Japanese FA patients...
  17. ncbi A heterozygous frameshift mutation in the Fanconi anemia C gene in familial T-ALL and secondary malignancy
    J R Rischewski
    University Children s Hospital, Hamburg, Germany
    Klin Padiatr 212:174-6. 2000
    ..We are currently screening a pediatric population with hematologic malignancies for mutations in the FANCA, FANCC and FANCG gene, and report here on siblings carrying a heterozygous frameshift mutation in the FANCC Gene.
  18. doi Validation of Fanconi anemia complementation Group A assignment using molecular analysis
    Nabil N Moghrabi
    Department of Molecular and Medical Genetics, Clinical Genetics Laboratories, Oregon Health and Science University, 3181 S W Sam Jackson Park Road, Portland, OR 97239, USA
    Genet Med 11:183-92. 2009
    ..of this study was (1) confirmation of the accuracy of Fanconi anemia complementation group assignment to Group A (FANCA) and (2) development of a rapid mutation detection strategy that ensures the efficient capture of all FANCA ..
  19. pmc Preclinical correction of human Fanconi anemia complementation group A bone marrow cells using a safety-modified lentiviral vector
    P S Becker
    Division of Hematology, Department of Medicine, University of Washington, 815 Mercer Street, Seattle, WA 98109, USA
    Gene Ther 17:1244-52. 2010
    ..transduction of hematopoietic progenitor cells from patients with Fanconi anemia complementation group A (FANCA)...
  20. ncbi Targeted disruption of exons 1 to 6 of the Fanconi Anemia group A gene leads to growth retardation, strain-specific microphthalmia, meiotic defects and primordial germ cell hypoplasia
    Jasmine C Y Wong
    Program in Genetics and Genomics Biology, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Hum Mol Genet 12:2063-76. 2003
    ..To study the in vivo role of the FA group A gene (Fanca), gene-targeting techniques were used to generate Fanca(tm1Hsc) mice in which Fanca exons 1-6 were replaced by a ..
  21. ncbi Cloning and characterization of murine fanconi anemia group A gene: Fanca protein is expressed in lymphoid tissues, testis, and ovary
    H J van de Vrugt
    Department of Clinical Genetics and Human Genetics, Free University Medical Centre, Amsterdam, The Netherlands
    Mamm Genome 11:326-31. 2000
    ..We have cloned the murine homolog of FANCA, the gene mutated in the major FA complementation group (FA-A)...
  22. ncbi The Fanconi anemia group A protein modulates homologous repair of DNA double-strand breaks in mammalian cells
    Yun gui Yang
    International Agency for Research on Cancer IARC, 150 cours Albert Thomas, F 69008 Lyon, France
    Carcinogenesis 26:1731-40. 2005
    ..Here, we have investigated specifically the function of the FA group A protein (FANCA) in the repair of DSBs in mammalian cells...
  23. ncbi Expression of the Fanconi anemia group A gene (Fanca) during mouse embryogenesis
    R Abu-Issa
    Departments of Molecular and Human Genetics, Biochemistry, and Medicine, The Developmental Biology Program, Baylor College of Medicine, Houston, TX, USA
    Blood 94:818-24. 1999
    ..understand the relationship between these groups, we analyzed the expression pattern of the mouse FA group-A gene (Fanca) during embryogenesis and compared it with the known pattern of the group-C gene (Fancc)...
  24. ncbi The molecular biology of Fanconi anemia
    Hannah Tamary
    Institute of Hematology Oncology, Schneider Children s Medical Center of Israel, Pediatric Hematology Oncology Laboratory, Felsenstein Medical Research Center, Rabin Medical Center Beilinson Campus, Petah Tiqva, Israel
    Isr Med Assoc J 4:819-23. 2002
    ..Six genes (FANCA, FANCC, FANCD2, FANCE, FANGF, FANCG) have been cloned so far...
  25. ncbi Spectrum of FANCA mutations in Italian Fanconi anemia patients: identification of six novel alleles and phenotypic characterization of the S858R variant
    Maria Savino
    Servizio di Genetica Medica, IRCCS Ospedale CSS, San Giovanni Rotondo, Foggia, Italy
    Hum Mutat 22:338-9. 2003
    ..Here, we report six novel FANCA sequence changes and review all the mutations identified in Italy...
  26. ncbi Hsp90 regulates the Fanconi anemia DNA damage response pathway
    Tsukasa Oda
    Laboratory of Molecular Genetics, Department of Molecular and Cellular Biology, Institute of Molecular and Cellular Regulation, Gunma University, 3 39 15 Showa Machi, Maebashi, Gunma 371 8512, Japan
    Blood 109:5016-26. 2007
    ..In the FA pathway, assembly of 8 FA proteins including FANCA into a nuclear multiprotein complex, and the complex-dependent activation of FANCD2 are critical events for ..
  27. ncbi Abnormalities in liver iron accumulation during N-2-fluorenylacetamide hepatocarcinogenesis that are dependent or independent of continued carcinogen action
    K Furuya
    Naylor Dana Institute for Disease Prevention, American Health Foundation, Valhalla, NY 10595, USA
    Toxicol Pathol 12:136-42. 1984
    The characteristics of liver iron accumulation were studied during N-2-fluorenylacetamide (FAA)-induced hepatocarcinogenesis in rats...
  28. ncbi Anthelmintic efficacy of genistein, the active principle of Flemingia vestita (Fabaceae): alterations in the free amino acid pool and ammonia levels in the fluke, Fasciolopsis buski
    Pradip Kumar Kar
    Department of Zoology, North Eastern Hill University, Shillong 793022, India
    Parasitol Int 53:287-91. 2004
    ..The amino acid composition of F. buski was demonstrated using HPLC and it was observed that the free amino acid (FAA) pool of the control worm consisted of aspartate, threonine, serine, glutamic acid, glutamine, proline, glycine, ..
  29. ncbi 5,6-dimethylxanthenone-4-acetic acid (DMXAA): a new biological response modifier for cancer therapy
    Shufeng Zhou
    Division of Pharmacology and Clinical Pharmacology, Faculty of Medical and Health Sciences, University ofAuckland, New Zealand
    Invest New Drugs 20:281-95. 2002
    ..Preclinical studies indicated that DMXAA had more potent anti-tumour activity compared to flavone-8-acetic acid (FAA)...
  30. ncbi Comparison of the reactivity of tetradecenoic acids, a triacsin, and unsaturated oximes with four purified Saccharomyces cerevisiae fatty acid activation proteins
    L J Knoll
    Department of Molecular Biology and Pharmacology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 270:20090-7. 1995
    ..Saccharomyces cerevisiae contains at least five acyl-CoA synthetases (fatty acid activation proteins, or Faaps). Four FAA genes have been recovered to date...
  31. doi Histological and clinical findings in different surgical strategies for focal axillary hyperhidrosis
    Falk G Bechara
    Department of Dermatology and Allergology, Ruhr University Bochum, Bochum, Germany
    Dermatol Surg 34:1001-9; discussion 1009. 2008
    Although a variety of different surgical strategies for focal axillary hyperhidrosis (FAH) have proven effective, little is known of intraoperative and postoperative histologies of different surgical methods.
  32. ncbi Composition of the excitatory drive during swimming in two amphibian embryos: Rana and Bufo
    R Perrins
    School of Biological Sciences, University of Bristol, UK
    J Comp Physiol A 179:563-73. 1996
    ..applications of antagonists show that presumed motoneurons in Rana and Bufo embryos receive both cholinergic and FAA input during swimming...
  33. ncbi Increased plasma serotonin following treatment with flavone-8-acetic acid, 5,6-dimethylxanthenone-4-acetic acid, vinblastine, and colchicine: relation to vascular effects
    B C Baguley
    Cancer Research Laboratory, University of Auckland School of Medicine, New Zealand
    Oncol Res 9:55-60. 1997
    ..These agents include flavone acetic acid (FAA), an antitumor agent with high experimental but no clinical antitumor activity, 5,6-dimethylxanthenone-4-acetic ..
  34. ncbi Comparison and cross-species expression of the acetyl-CoA synthetase genes of the Ascomycete fungi, Aspergillus nidulans and Neurospora crassa
    I F Connerton
    Department of Genetics, University of Cambridge, UK
    Mol Microbiol 4:451-60. 1990
    ..acetate-inducible enzyme acetyl-coenzyme A synthetase from Neurospora crassa and Aspergillus nidulans (acu-5 and facA, respectively) have been cloned and their sequences compared...
  35. ncbi Prevalence of drugs and alcohol in fatal civil aviation accidents between 1994 and 1998
    D V Canfield
    Civil Aeromedical Institute, Federal Aviation Administration, Oklahoma City, OK 73125, USA
    Aviat Space Environ Med 72:120-4. 2001
    The use of drugs and alcohol in aviation is closely monitored by the FAA Office of Aviation Medicine's (OAM's) Civil Aeromedical Institute (CAMI) through the toxicological analysis of specimens from pilots who have died in aviation ..
  36. ncbi Chemical composition of inks of diverse marine molluscs suggests convergent chemical defenses
    Charles D Derby
    Department of Biology, Brains and Behavior Program and Center for Behavioral Neuroscience, Georgia State University, Atlanta, Georgia 30302 4010, USA
    J Chem Ecol 33:1105-13. 2007
    ..The latter two mechanisms are facilitated by millimolar concentrations of free amino acids (FAA) in sea hare ink and opaline, which stimulate the chemosensory systems of predators, ultimately leading to escape ..
  37. ncbi Communication towers, lights, and birds: successful methods of reducing the frequency of avian collisions
    Joelle Gehring
    Central Michigan University, Department of Biology, Mount Pleasant, Michigan 48859, USA
    Ecol Appl 19:505-14. 2009
    ..To determine the relative collision risks that different nighttime Federal Aviation Administration (FAA) communication tower obstruction lighting systems pose to night-migrating birds, we compared fatalities at towers ..
  38. ncbi Metabolite profile in milk of lactating rats after treatment with a carcinogen, N-2-fluorenylacetamide
    D Malejka-Giganti
    Laboratory for Cancer Research, Veterans Administration Medical Center, Minneapolis, MN 55417
    Drug Metab Dispos 15:760-6. 1987
    ..in milk and urine of lactating rats 4 to 5 hr after each of 3 to 6 ip injections of N-2-fluorenylacetamide (2-FAA) at 0.2 mmol/kg of body weight...
  39. doi Mammary uptake, portal-drained visceral flux, and hepatic metabolism of free and peptide-bound amino acids in cows fed steam-flaked or dry-rolled sorghum grain diets
    H Tagari
    Department of Animal Sciences, Faculty of Agricultural, Food and Environmental Quality Sciences, The Hebrew University of Jerusalem, Rehovot, Israel
    J Dairy Sci 91:679-97. 2008
    Our objectives were to measure net fluxes of free AA (FAA) and peptide-bound AA (PBAA) across portal-drained viscera, liver, splanchnic tissues, and mammary tissues, and milk AA output of lactating Holstein cows (n = 8, 86 +/- 8 d in ..
  40. doi Intra- and extracellular osmotic regulation in the hololimnetic Caridea and Anomura: a phylogenetic perspective on the conquest of fresh water by the decapod Crustacea
    Samuel Coelho de Faria
    Departamento de Biologia, Faculdade de Filosofia, Ciencias e Letras de Ribeirao Preto, Universidade de Sao Paulo, Ribeirao Preto, SP, 14040 901, Brazil
    J Comp Physiol B 181:175-86. 2011
    ..Muscle total free amino acids (FAA) increase slowly but markedly in response to the rapid rise in hemolymph osmolality consequent to hyperosmotic ..
  41. ncbi Fatty acid ethyl and methyl ester synthases, and fatty acid anilide synthase in HepG2 and AR42J cells: interrelationships and inhibition by tri-o-tolyl phosphate
    B S Kaphalia
    Department of Pathology, University of Texas Medical Branch, Galveston 77555, USA
    Toxicol Appl Pharmacol 159:134-41. 1999
    ..Approximately 86-90% inhibition of FAEE, FAME, and FAA synthesizing activities was found in HepG2 cells following exposure to 2.5 microM TOTP...
  42. ncbi Adaptive shifts in osmoregulatory strategy and the invasion of freshwater by brachyuran crabs: evidence from Dilocarcinus pagei (Trichodactylidae)
    Alessandra Augusto
    Departamento de Biologia, Universidade de Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil
    J Exp Zool A Ecol Genet Physiol 307:688-98. 2007
    ..pagei does not produce dilute urine. Total free amino acid (FAA) concentrations in embryos (14.9+/-1.2), juveniles (32.8+/-0.1) and adult muscle (10.9+/-2...
  43. ncbi Apparent ruminal degradation and rumen escape of soluble nitrogen fractions in grass and grass silage administered intraruminally to lactating dairy cows
    H Volden
    Department of Animal Science, Agricultural University of Norway, As
    J Anim Sci 80:2704-16. 2002
    ..Soluble protein and long-chain peptides (PLP), small peptides (SP) and free AA (FAA) were obtained from fresh grass and grass silages fertilized with different levels of N...
  44. ncbi Free amino acid composition in juveniles of Mytilus galloprovincialis: spatial variability after Prestige oil spill
    Jose M F Babarro
    Instituto de Investigaciones Marinas CSIC, Eduardo Cabello 6, 36208 Vigo, Spain
    Comp Biochem Physiol A Mol Integr Physiol 145:204-13. 2006
    Composition of free amino acids (FAA) in juveniles of Mytilus galloprovincialis was analysed along a large geographical coastline area in Galicia (NW Spain)...
  45. ncbi L-thyroxine (T4) elevates the free amino acid pool of haemolymph plasma of tasar silkworm, Antheraea mylitta drury (Lepidoptera: Saturniidae)
    K D Reddy
    Insect Physiology Laboratory, Central Tasar Research and Training Institute, Nagri, Ranchi, India
    Horm Metab Res 26:570-3. 1994
    Concentration of free amino acids (FAA) in the haemolymph plasma of male and female tasar silkworm, A. mylitta, during fifth larval instar were determined by amino acid analyzer after administration of vertebrate thyroxine (T4)...
  46. ncbi Complementation of Saccharomyces cerevisiae strains containing fatty acid activation gene (FAA) deletions with a mammalian acyl-CoA synthetase
    L J Knoll
    Department of Molecular Biology and Pharmacology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 270:10861-7. 1995
    Four unlinked fatty acid activation (FAA) genes encoding acyl-CoA synthetases have been identified in Saccharomyces cerevisiae and characterized by noting the phenotypes of isogenic strains containing all possible combinations of faa ..
  47. ncbi Metabolism of the carcinogen N-hydroxy-N-2-fluorenylacetamide by rat peritoneal neutrophils
    D Malejka-Giganti
    Veterans Affairs Medical Center, Minneapolis, MN 55417
    Carcinogenesis 14:341-6. 1993
    The in vitro metabolism of a locally carcinogenic N-hydroxy-N-2-fluorenylacetamide (N-OH-2-FAA) by rat peritoneal polymorphonuclear leukocytes (PMNL), chiefly neutrophils, elicited with intraperitoneal injections of proteose peptone, was ..
  48. ncbi Crystal structure and mechanism of a carbon-carbon bond hydrolase
    D E Timm
    Department of Biochemistry and Molecular Biology Indiana University School of Medicine 635 Barnhill Drive, Indianapolis, Indiana 46202, USA
    Structure 7:1023-33. 1999
    Fumarylacetoacetate hydrolase (FAH) catalyzes the final step of tyrosine and phenylalanine catabolism, the hydrolytic cleavage of a carbon-carbon bond in fumarylacetoacetate, to yield fumarate and acetoacetate...
  49. ncbi Free and peptide amino acid net flux across the rumen and the mesenteric- and portal-drained viscera of sheep
    D Remond
    Unite de Recherches sur les Herbivores, Institut National de la Recherche Agronomique de Clermont Ferrand, St Genes Champanelle, France
    J Anim Sci 78:1960-72. 2000
    ..Control and postinjection net fluxes of plasma free amino acids (FAA) and PAA were determined...
  50. ncbi Inflight cabin ozone aboard long duration C-5 airlift missions: a historical issue revisited
    S M Hetrick
    U S Air Force Institute of Aerospace Medicine, Residency in Aerospace Medicine, Brooks AFB, TX, USA
    Aviat Space Environ Med 71:408-14. 2000
    ..occasionally exposed to ambient, stratospheric ozone concentrations in excess of Federal Aviation Administration (FAA) ceiling and time-weighted average (TWA) standards...
  51. ncbi Should consensus be 'the commission method' in the US? The perspective of the Federal Advisory Committee Act, regulations, and case law
    Bethany Spielman
    Southern Illinois University School of Medicine, Box 19603, Springfield, IL, USA
    Bioethics 17:341-56. 2003
    ..Specifically, the paper examines the Federal Advisory Committee Act (FACA)--the statute, its regulations, and case law...
  52. ncbi Toxicological findings from 1587 civil aviation accident pilot fatalities, 1999-2003
    Arvind K Chaturvedi
    Bioaeronautical Sciences Research Laboratory AAM 610, FAA Civil Aerospace Medical Institute, P O Box 25082, Oklahoma City, OK 73125 5066, USA
    Aviat Space Environ Med 76:1145-50. 2005
    The prevalence of drug and ethanol use in aviation is monitored by the Federal Aviation Administration (FAA)...
  53. doi Friedmanniella luteola sp. nov., Friedmanniella lucida sp. nov., Friedmanniella okinawensis sp. nov. and Friedmaniella sagamiharensis sp. nov., isolated from spiders
    Kimika Iwai
    Innovative Drug Research Laboratories, Kyowa Hakko Kirin Co, Ltd 3 6 6, Asahi machi, Machida shi, Tokyo 194 8533, Japan
    Int J Syst Evol Microbiol 60:113-20. 2010
    ..Two strains (FA1(T) and FA2(T)) formed a cluster together with Friedmanniella capsulata and Friedmanniella lacustris and the other ..
  54. pmc Saccharomyces cerevisiae contains four fatty acid activation (FAA) genes: an assessment of their role in regulating protein N-myristoylation and cellular lipid metabolism
    D R Johnson
    Department of Molecular Biology and Pharmacology, Washington University School of Medicine, St Louis, Missouri 63110
    J Cell Biol 127:751-62. 1994
    ..We have recently isolated three unlinked Fatty Acid Activation (FAA) genes encoding long chain acylCoA synthetases and have now recovered a fourth by genetic complementation...
  55. ncbi A role for Saccharomyces cerevisiae fatty acid activation protein 4 in regulating protein N-myristoylation during entry into stationary phase
    K Ashrafi
    Department of Molecular Biology and Pharmacology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 273:25864-74. 1998
    ..These strains contained either a wild type or mutant NMT1 allele, and wild type or null alleles of each FAA. Only the combination of the Nmt mutant (nmt451Dp; reduced affinity for myristoyl-CoA) and loss of Faa4p produced a ..
  56. ncbi Animal models of tyrosinemia
    Kimitoshi Nakamura
    Department of Pediatrics, Kumamoto University Graduate School of Medical Science, Kumamoto 860 8556, Japan
    J Nutr 137:1556S-1560S; discussion 1573S-1575S. 2007
    ..HT I is due to mutations in the fumarylacetoacetate hydrolase (Fah) gene, which encodes the last enzyme in the tyrosine catabolic pathway...
  57. ncbi The influence of MK-801 on the hippocampal free arachidonic acid level and Na+,K+-ATPase activity in global cerebral ischemia-exposed rats
    Jasenka Mršić-Pelčić
    Department of Pharmacology, School of Medicine, University of Rijeka, Brace Branchetta 20 1, 51000 Rijeka, Croatia
    Prog Neuropsychopharmacol Biol Psychiatry 26:1319-26. 2002
    The influence of 20 min global cerebral ischemia on the free arachidonic acid (FAA) level and Na+,K+-ATPase activity in the rat hippocampus at different time points after ischemia was examined...
  58. pmc Ploidy reductions in murine fusion-derived hepatocytes
    Andrew W Duncan
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon, United States of America
    PLoS Genet 5:e1000385. 2009
    We previously showed that fusion between hepatocytes lacking a crucial liver enzyme, fumarylacetoacetate hydrolase (FAH), and wild-type blood cells resulted in hepatocyte reprogramming...
  59. doi Vanadium complexes having [V(IV)O](2+) and [V(V)O(2)](+) cores with binucleating dibasic tetradentate ligands: Synthesis, characterization, catalytic and antiamoebic activities
    Mannar R Maurya
    Department of Chemistry, Indian Institute of Technology Roorkee, Roorkee, 247 667, India
    Dalton Trans 39:1345-60. 2010
    Binucleating hydrazones CH(2)(H(2)sal-bhz)(2) (I) and CH(2)(H(2)sal-fah)(2) (II), derived from 5,5'-methylbis(salicylaldehyde) and benzoylhydrazide or 2-furoylhydrazide, react with [V(IV)O(acac)(2)] to give dinuclear V(IV)O-complexes [CH(..
  60. ncbi A potential biomarker of kidney damage identified by proteomics: preliminary findings
    Lasantha R Bandara
    Oxford GlycoSciences UK Ltd, The Forum, 86 Milton Park, Abingdon, Oxon, OX14 4RY, UK
    Biomarkers 8:272-86. 2003
    ..One toxicity-associated plasma protein was identified as the cellular enzyme fumarylacetoacetate hydrolase (FAH), which is known to be required for tyrosine metabolism...
  61. pmc Modulations of hepatic gamma-glutamyltranspeptidase and N-hydroxy-N-2-fluorenylacetamide sulfotransferase activities following treatment of rats with a hepatocarcinogenic regimen: effect of partial hepatectomy
    P Vijayaraghavan
    Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis
    Environ Health Perspect 102:105-8. 1994
    ..assays of gamma-glutamyltranspeptidase (gamma-GTP) and N-hydroxy-N-2-fluorenylacetamide sulfotransferase (N-OH-2-FAA ST) activities to monitor the effects of treatment of male Sprague-Dawley rats with a two-stage hepatocarcinogenic ..
  62. ncbi Combining bioreductive drugs (SR 4233 or SN 23862) with the vasoactive agents flavone acetic acid or 5,6-dimethylxanthenone acetic acid
    S Cliffe
    Department of Pathology, University of Auckland School of Medicine, New Zealand
    Int J Radiat Oncol Biol Phys 29:373-7. 1994
    To determine whether 5,6-dimethylxanthenone acetic acid (DMXAA), a potent analogue of flavone acetic acid (FAA) inhibits blood flow in mouse mammary tumors, and to assess whether DMXAA enhances the antitumor effects of Tirapazamine (SR ..
  63. ncbi Screening strategies for a highly polymorphic gene: DHPLC analysis of the Fanconi anemia group A gene
    J Rischewski
    Department of Paediatric Haematology and Oncology, University Childrens Hospital, Martinistr 52, D 20246, Hamburg, Germany
    J Biochem Biophys Methods 47:53-64. 2001
    Patients with Fanconi anemia (Fanc) are at risk of developing leukemia. Mutations of the group A gene (FancA) are most common. A multitude of polymorphisms and mutations within the 43 exons of the gene are described...
  64. ncbi Frequent mutation reversion inversely correlates with clinical severity in a genetic liver disease, hereditary tyrosinemia
    Sylvie I Demers
    Laboratory of Cellular and Developmental Genetics, Department of Medicine, Université Laval and CHUL Research Center, CHUQ, Ste Foy, Quebec, Canada
    Hum Pathol 34:1313-20. 2003
    ..HTI), a severe disease affecting primarily the liver, is caused by a deficiency of fumarylacetoacetate hydrolase (FAH). HTI is clinically heterogeneous, with no correlation between genotype and phenotype...
  65. pmc Gonadotropin-releasing hormone regulates expression of the DNA damage repair gene, Fanconi anemia A, in pituitary gonadotroph cells
    Rachel Larder
    Human Reproductive Sciences Unit, Centre for Reproductive Biology, The University of Edinburgh Chancellors Building, Edinburgh EH16 4SB, UK
    Biol Reprod 71:828-36. 2004
    ..This approach identified Fanconi anemia a (Fanca), a gene implicated in DNA damage repair, as a differentially expressed transcript...
  66. ncbi Pharmaceuticals in the river Elbe and its tributaries
    S Wiegel
    Bundesamt für Verbraucherschutz und Lebensmittelsicherheit, Messeweg 11 12, 38104 Braunschweig, Germany
    Chemosphere 57:107-26. 2004
    ..The metamizole metabolites N-acetyl-4-aminoantipyrine (AAA) and N-formyl-4-aminoantipyrine (FAA) were found in concentrations from <20 to 939 ng/l...
  67. ncbi Phosphorylation of fanconi anemia (FA) complementation group G protein, FANCG, at serine 7 is important for function of the FA pathway
    Fengyu Qiao
    Departments of Microbiology and Pediatrics, University of Virginia Health System, Charlottesville, Virginia 22908, USA
    J Biol Chem 279:46035-45. 2004
    ..Unexpectedly, FANCG(S7A) bound to and stabilized the endogenous forms of the FANCA and FANCC proteins in the FA-G cells...
  68. ncbi Ozone exposures during trans-continental and trans-Pacific flights
    J D Spengler
    Exposure, Epidemiology and Risk Program, Department of Environmental Health, Harvard School of Public Health, Boston, MA 02215, USA
    Indoor Air 14:67-73. 2004
    ..p.b. (30.1). Twenty percent of the measurements exceeded 100 p.p.b., the FAA-recommended level. Eleven percent of the measurements exceeded 120 p.p.b...
  69. ncbi A common founder mutation in FANCA underlies the world's highest prevalence of Fanconi anemia in Gypsy families from Spain
    Elsa Callen
    Universitat Autònoma de Barcelona and the Hospital Meterno Infantil Vall d Hebron, Barcelona, Spain
    Blood 105:1946-9. 2005
    ..DNA sequencing of the FANCA gene in 8 unrelated Spanish Gypsy FA families after retroviral subtyping revealed a homozygous FANCA mutation (..
  70. ncbi Identification of hypertension-related genes through an integrated genomic-transcriptomic approach
    Chana Yagil
    Israel Rat Genome Center and Laboratory for Molecular Medicine, Department of Nephrology and Hypertension, Faculty of Health Sciences, Barzilai Medical Center Campus of the Ben Gurion University, Ashkelon, Israel
    Circ Res 96:617-25. 2005
    ..RT-PCR confirmed 7 genes, leaving TcTex1, Myadm, Lisch7, Axl-like, Fah, PRC1-like, and Serpinh1. None of these genes has been implicated in hypertension before...
  71. ncbi A practical guide to social networks
    Rob Cross
    University of Virginia, McIntire School of Commerce, Charlotteville, USA
    Harv Bus Rev 83:124-32, 150. 2005
    ..Drawing on examples from Novartis, the FAA, and Sallie Mae, the authors offer managers the tools they need to determine which network will deliver the best ..
  72. pmc Evidence for subcomplexes in the Fanconi anemia pathway
    Annette L Medhurst
    Department of Clinical Genetics and Human Genetics, Vrije Universiteit University Medical Center, Van der Boechorststraat 7, 1081 BT Amsterdam, The Netherlands
    Blood 108:2072-80. 2006
    ..We demonstrate a direct interaction between FANCB and FANCL and that a complex of these proteins binds FANCA. The interaction between FANCA and FANCL is dependent on FANCB, FANCG, and FANCM, but independent of FANCC, FANCE, ..
  73. pmc The liver of woodchucks chronically infected with the woodchuck hepatitis virus contains foci of virus core antigen-negative hepatocytes with both altered and normal morphology
    Chunxiao Xu
    Division of Basic Science, Fox Chase Cancer Center, 333 Cottman Avenue, Philadelphia, PA 19111, USA
    Virology 359:283-94. 2007
    ..chronically infected with woodchuck hepatitis virus (WHV) contain foci of morphologically altered hepatocytes (FAH) with "basophilic", "amphophilic" and "clear cell" phenotypes, which are possibly pre-..
  74. ncbi Does fluctuating thermal regime trigger free amino acid production in the parasitic wasp Aphidius colemani (Hymenoptera: Aphidiinae)?
    Hervé Colinet
    Unité d Ecologie et de Biogéographie, Biodiversity Research Centre, Universite Catholique de Louvain, Louvain la Neuve, Belgium
    Comp Biochem Physiol A Mol Integr Physiol 147:484-92. 2007
    ..the initiation of a metabolic response involving synthesis of cryoprotective compounds, such as free amino acids (FAA)...
  75. ncbi The ability of coumarin-, flavanon- and flavonol-analogues of flavone acetic acid to stimulate human monocytes
    Mariagnese Barbera
    Department of Pharmacology and Anaesthesiology, University of Padova, 35131 Padova, Italy
    Oncol Rep 19:187-96. 2008
    Flavone acetic acid (FAA) is a semi-synthetic flavonoid characterised by potent immune-modulatory and antivascular activity on mice but not in humans...
  76. doi Occurrence, fate and assessment of polar metamizole (dipyrone) residues in hospital and municipal wastewater
    Dirk F Feldmann
    Institute of Food Chemistry, Technical University Berlin, Sekr TIB4 3 1, Gustav Meyer Allee 25, 13355 Berlin, Germany
    Chemosphere 71:1754-64. 2008
    ..The loads of the metabolites aminoantipyrin (AA), 4-acetylaminoantipyrin (AAA) and 4-formyl-aminoantipyrin (FAA), rapidly formed after the application of metamizole, were predicted from pharmacokinetic data and based on the ..
  77. doi Time-imposed daily restricted feeding induces rhythmic expression of Fgf21 in white adipose tissue of mice
    Katsutaka Oishi
    Biological Clock Research Group, Institute for Biological Resources and Functions, National Institute of Advanced Industrial Science and Technology, Tsukuba, Ibaraki, Japan
    Biochem Biophys Res Commun 412:396-400. 2011
    ..The free-running period of locomotor activity rhythm under ALF and the food anticipatory activity (FAA) under RF remained intact in Fgf21 knockout (KO) mice, suggesting that FGF21 is dispensable for both the central ..
  78. doi Effects of medium-voltage electrical stimulation on postmortem changes in fat-tailed sheep
    Maryam Abbasvali
    Department of Food Hygiene and Public Health, School of Veterinary Medicine, Shiraz University, Shiraz, Iran
    J Food Sci 77:S47-53. 2012
    ..05). The results of free amino acid (FAA) content and myofibrillar fragmentation index (MFI) revealed a significant improvement of proteolysis and ..
  79. pmc Proteomic profiling of differentially expressed proteins from Bax inhibitor-1 knockout and wild type mice
    Bo Li
    Department of Pharmacology and Cardiovascular Research Center, Chonbuk National University, Jeonju 561 182, Korea
    Mol Cells 34:15-23. 2012
    ..protein 75 (GRP75/mortalin/ PBP74/mthsp70), peroxiredoxin6 (Prx6) and fumarylacetoacetate hydrolase (FAH) showed a pI shift that could be attributed to post-translational modifications...
  80. ncbi Compound mutations (R237X and L375P) in the fumarylacetoacetate hydrolase gene causing tyrosinemia type I in a Chinese patient
    Yan Yan Cao
    Department of Medical Genetics, Capital Institute of Pediatrics, Beijing 100020, China
    Chin Med J (Engl) 125:2132-6. 2012
    Mutations in fumarylacetoacetate hydrolase (FAH) gene can lead to tyrosinemia type 1 (HT1), a relatively rare autosomal recessive disorder. To date, no molecular genetic defects of HT1 in China have been described...
  81. ncbi Aerial organ anatomy of Smilax syphilitica (Smilacaceae)
    João Marcelo Silva
    Departmento de Ciencias Biologicas, Escola Superior de Agricultura Luiz de Queiroz, Universidade de Sao Paulo, 13418 900, Piracicaba, SP, Brazil
    Rev Biol Trop 60:1137-48. 2012
    ..For this, a total of three samples of Smilax were collected per site. Sample leaves and stems were fixed with FAA 50, embedded in historesin, sectioned on a rotary microtome, stained and mounted in synthetic resin...
  82. doi Reversal of hepatocyte senescence after continuous in vivo cell proliferation
    Min Jun Wang
    Department of Cell Biology, Second Military Medical University, Shanghai, P R China Center for Stem Cell and Medicine, Graduate School, Second Military Medical University, Shanghai, P R China
    Hepatology 60:349-61. 2014
    ..Taking advantage of serial hepatocyte transplantation assays in the fumarylacetoacetate hydrolase-deficient (Fah(-/-)) mouse, we studied the senescence of hepatocytes that had undergone continuous cell proliferation over a long ..
  83. doi Characterization and quantitative amino acids analysis of analgesic peptides in cinobufacini injection by size exclusion chromatography, matrix-assisted laser desorption/ionization time of flight mass spectrometry and gas chromatography mass spectrometry
    Xu Wu
    Institute of Chinese Materia Medica, China Academy of Chinese Medical Sciences, Beijing, 100700, China
    Biomed Chromatogr 29:138-47. 2015
    ..by gas chromatography mass spectrometry (GC-MS) was developed to determine both free and combined amino acids (FAA and CAA) in cinobufacini injection. This method achieved good linearity (R(2) , 0.9909-0...
  84. doi Extensive double humanization of both liver and hematopoiesis in FRGN mice
    Elizabeth M Wilson
    Yecuris Corp, Tigard, OR, USA
    Stem Cell Res 13:404-12. 2014
    ..Immune-deficient, fumarylacetoacetate hydrolase (Fah(-/-)), Rag2(-/-) and Il2rg(-/-) deficient animals on the NOD-strain background (FRGN) were simultaneously co-..
  85. doi Smad7 regulates compensatory hepatocyte proliferation in damaged mouse liver and positively relates to better clinical outcome in human hepatocellular carcinoma
    Teng Feng
    Molecular Hepatology Alcohol Associated Diseases, Dept of Medicine II, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
    Clin Sci (Lond) 128:761-74. 2015
    ..Transgenic hepatocyte-specific Smad7 overexpression in damaged liver of fumarylacetoacetate hydrolase (FAH)-deficient mice increased compensatory proliferation of hepatocytes...
  86. doi Therapeutic effects of growth hormone combined with low-dose stanozolol on growth velocity and final height of girls with Turner syndrome
    Hui Xiong
    Pediatric Department, The First Affiliated Hospital, Sun Yat Sen University, Guangzhou, China
    Clin Endocrinol (Oxf) 83:223-8. 2015
    ..This study aimed to investigate the therapeutic effects of recombinant human growth hormone (rhGH) combined with low-dose stanozolol on the growth and final adult height (FAH) of girls with Turner syndrome (TS).
  87. doi High-resolution MS and MS(n) investigation of ozone oxidation products from phenazone-type pharmaceuticals and metabolites
    Maxime Favier
    KU Leuven, Department of Pharmaceutical and Pharmacological Sciences, Pharmaceutical Analysis, Herestraat 49, 3000 Leuven, Belgium
    Chemosphere 136:32-41. 2015
    ..Acetamido antipyrine (AAA) and formyl aminoantipyrine (FAA) - the main metabolites of aminopyrine and metamizole - have also been detected in sub μg L(-1) concentrations in ..
  88. doi Clinical aspects of Fanconi anemia individuals with the same mutation of FANCF identified by next generation sequencing
    Elena Nicchia
    Department of Medical Sciences, University of Trieste, Trieste, Italy
    Birth Defects Res A Clin Mol Teratol 103:1003-10. 2015
    ..FA is genetically heterogeneous as it is caused by at least 17 different genes. Among these, FANCA, FANCC, and FANCG account for approximately 85% of the patients whereas the remaining genes are mutated in only a ..
  89. doi Food reward without a timing component does not alter the timing of activity under positive energy balance
    V van der Vinne
    Chronobiology Unit, Groningen Institute for Evolutionary Life Sciences, University of Groningen, Groningen, The Netherlands Electronic address
    Neuroscience 304:260-5. 2015
    ..food access is restricted to a few hours during the resting (light) phase resulting in food anticipatory activity (FAA) in the hours preceding food availability...
  90. ncbi Lentiviral-Mediated Gene Therapy in Fanconi Anemia-A Mice Reveals Long-Term Engraftment and Continuous Turnover of Corrected HSCs
    F Javier Molina-Estevez
    CIEMAT, Edificio 70, Avenida Complutense 40, 28040 Madrid Spain
    Curr Gene Ther 15:550-62. 2015
    ..anemia, we have carried out a genome-wide screening of lentiviral insertion sites after the gene correction of Fanca(-/-) hematopoietic stem cells (HSCs), using LAM-PCR and 454-pyrosequencing...
  91. doi All-trans-retinoic acid and retinol binding to the FA1 site of human serum albumin competitively inhibits heme-Fe(III) association
    Elena Di Muzio
    Department of Sciences, Roma Tre University, I 00146 Roma, Italy
    Arch Biochem Biophys 590:56-63. 2016
    ..All-trans-RA and all-trans-ROL binding to the FA1 site of HSA competitively inhibit heme-Fe(III) association...
  92. doi Leaching Behavior of Selected Trace and Toxic Metals in Coal Fly Ash Samples Collected from Two Thermal Power Plants, India
    P Sandeep
    Environmental Monitoring and Assessment Section, Bhabha Atomic Research Centre, Trombay, Mumbai, 400085, India
    Bull Environ Contam Toxicol 97:425-31. 2016
    ..In the present study, leaching behavior of metals (As, Se, Cr, Pb, V, Zn, etc.) in two different FA samples (FA1 and FA2) was investigated at various pH (2-12), temperatures of leachate solution and using TCLP...
  93. doi β-Cristobalite (001) surface as 4-formaminoantipyrine adsorbent: First principle study of the effect on adsorption of surface modification
    S Simonetti
    IFISUR, Universidad Nacional del Sur, CONICET, Departamento de Física UNS, Bahia Blanca, Argentina Universidad Tecnológica Nacional UTN, Bahia Blanca, Argentina Electronic address
    Colloids Surf B Biointerfaces 148:287-292. 2016
    ..The role of surface modification on the features of 4-formaminoantipyrine (FAA) adsorbed on β-cristobalite (001) surface is studied by means of simulations based on the Density Functional ..
  94. pmc Severity of experimental traumatic brain injury modulates changes in concentrations of cerebral free amino acids
    Angela Maria Amorini
    Institute of Biochemistry and Clinical Biochemistry, Catholic University of Rome, Rome, Italy
    J Cell Mol Med . 2016
    In this study, concentrations of free amino acids (FAA) and amino group containing compounds (AGCC) following graded diffuse traumatic brain injury (mild TBI, mTBI; severe TBI, sTBI) were evaluated...
  95. pmc V(D)J recombination process and the Pre-B to immature B-cells transition are altered in Fanca-/- mice
    Thuy Vy Nguyen
    CNRS UMR 8200 Gustave Roussy, Villejuif, France
    Sci Rep 6:36906. 2016
    ..process, we sought to determine the impact of the FANC pathway on the Ig diversification process using Fanca-/- mice...
  96. doi Mechanism of Ubiquitination and Deubiquitination in the Fanconi Anemia Pathway
    Sylvie van Twest
    Genome Stability Unit, St Vincent s Institute of Medical Research, Fitzroy, VIC 3065, Australia
    Mol Cell 65:247-259. 2017
    ..b>FANCA and FANCG are dispensable for maximal in vitro ubiquitination...
  97. ncbi In vivo correction of murine tyrosinemia type I by DNA-mediated transposition
    Eugenio Montini
    Department of Medical and Molecular Genetics, Oregon Health and Sciences University, Portland, Oregon 97239, USA
    Mol Ther 6:759-69. 2002
    ..of the SB transposon in vivo by analyzing the expression and integration of a fumarylacetoacetate hydrolase (FAH) transposon in FAH-deficient mice...
  98. pmc Lessons learned from poisoning cases caused by 2 illegal rodenticides: Tetramine and fluoroacetamide
    Rongshuai Wang
    aDepartment of Forensic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan bInstitute of Forensic Science, Fujian Provincial Department of Public Security, Fuzhou, China
    Medicine (Baltimore) 95:e5103. 2016
    Tetramine (tetramethylene disulphotetramine, TETS) and fluoroacetamide (FAA) are known as illegal rodenticides with high toxicity to animal species and human beings, which could lead to severe clinical features, including reduction of ..
  99. ncbi Hereditary tyrosinemia type 1: novel missense, nonsense and splice consensus mutations in the human fumarylacetoacetate hydrolase gene; variability of the genotype-phenotype relationship
    J K Ploos van Amstel
    DNA Laboratory, Clinical Genetics Center, Utrecht, The Netherlands
    Hum Genet 97:51-9. 1996
    The complete fumarylacetoacetate hydrolase (FAH) genotype of probands of thirteen unrelated families with hereditary tyrosinemia type 1 (HT 1) was established...
  100. ncbi Intracellular localization of the Fanconi anemia complementation group A protein
    C E Walsh
    UNC Gene Therapy Center, University of North Carolina, Chapel Hill, North Carolina, 27599, USA
    Biochem Biophys Res Commun 259:594-9. 1999
    Mutations in the Fanconi anemia (FA) complementation group A (FANCA) gene leads to bone marrow failure, developmental abnormalities and cancer predisposition...
  101. ncbi Increases in tissue free amino acid levels in response to prolonged emersion in marine crabs: an ammonia-detoxifying process efficient in the intertidal Carcinus maenas but not in the subtidal Necora puber
    - Durand
    Equipe Ecophysiologie, Observatoire Océanologique de Roscoff UPMC, CNRS, INSU, Station Biologique, BP 74, F 29682 Roscoff Cedex, France
    J Exp Biol 202:2191-2202. 1999
    ..Changes in the free amino acid (FAA) content of their muscle, hepatopancreas and haemolymph were recorded during air-exposure and subsequent ..

Research Grants68

  1. Mice Humanized with Hepatocytes and iPS Cells from Patients with Metabolic Diseas
    Stephen C Strom; Fiscal Year: 2010
    ..These mice are immunodeficient and also deficient in the tyrosine catabolic enzyme, fumarylacetoacetate hydrolase (Fah -/-) and develop irreversible liver failure if left untreated...
  2. FANCD2 Monoubiquitination in DNA Damage Responses
    Paul R Andreassen; Fiscal Year: 2012
    ..FANCD2 monoubiquitination will be inhibited by shRNA-mediated suppression of FANCA. Also, mechanisms involved in the recruitment of FANCD2 to blocked replication forks will be elucidated using real-..
  3. Qishen Pang; Fiscal Year: 2015
    ..Using a FA knockout (Fanca) mouse model we have shown that preconditioning of Fanca-/- mice with CASIN allows significant engraftment of WT ..
  4. Lentiviral Vectors for the Treatment of Fanconi Anemia
    DAVID W CLAPP; Fiscal Year: 2010
    ..Specific Aim 1: Generate recombinant lentiviral constructs that encode the expression of the FANCA cDNA by the human phosphoglycerate kinase promoter or the elongation factor 1a promoter and test functional ..
  5. Double Humanization of Liver and Blood in Mice
    ELIZABETH MARIE WILSON; Fiscal Year: 2012
    ..Our company uses immune deficient fumarylacetoacetate hydrolase (Fah) knockout mice[2], bearing additional mutations in Rag2 and the interleukin common gamma chain receptor (Il2rg) ..
  6. FAH-Deficient Pigs
    Scott L Nyberg; Fiscal Year: 2011
    ..hereditary tyrosinemia type 1 (HT1) accomplished by knocking out the gene coding for fumarylacetoacetate hydrolase (FAH) in pigs...
  7. FAA AND FAD GENES AND PROTEINS
    Manuel Buchwald; Fiscal Year: 1999
    ..applicant shows now that, using the same expression cloning procedure he used to isolate FAC, he has isolated the FAA gene and confirmed its identity by the identification of mutations in FA-A cells; he also has a strong candidate ..
  8. Markus Grompe; Fiscal Year: 2016
    DESCRIPTION: Deficiency of fumarylacetoacetate hydrolase (FAH) causes tyrosinemia type I (hepatorenal tyrosinemia, HT1) in humans and is characterized by severe liver dysfunction, renal tubular damage and liver cancer...
  9. FANCONI ANEMIA--PATHOPHYSIOLOGY AND TREATMENT
    Christopher Walsh; Fiscal Year: 1999
    ..Recently, the gene responsible for FA-A was identified and cloned. A retroviral vector carrying the FAA cDNA was constructed as well as a packaging cell line to produce the vector. The retroviral vector, FAA5...
  10. YPE PETER DE JONG; Fiscal Year: 2015
    ..and of PKR activation in primary human hepatocytes, and further study these pathways in a new mouse model based on FAH- deficient liver injury mice...
  11. Symposium: Progress in Fanconi Anemia Research
    Grover Bagby; Fiscal Year: 2001
    ..The genes encoding the groups A (FANCA), C (FANCC), G (FANCG), D (FANCD), and F (FANCF) have been cloned...
  12. MOLECULAR ANALYSIS OF FANCONI'S ANEMIA C PROTEIN
    Sharon Plon; Fiscal Year: 2003
    ..b>FANCA is homologous to peroxidases, interacts with FANCG, and functions in the nucleus...
  13. Gene therapy for Fanconi anemia
    Hans Peter Kiem; Fiscal Year: 2009
    ..Thus, we propose to 1) complete preclinical studies of FANCA and FANCC lentiviral vectors in human FA cells and in FANCA-/- and FABCC-/- mice and optimize procedures for ..
  14. Gene Transfer of Hematopoietic Stem Cells
    Christopher Walsh; Fiscal Year: 2004
    ..Our strategy is to isolate and transduce Fanconi anemia complementation group A (FANCA) and C (FANCC) knockout mice primitive stem cells...
  15. Michael Karin; Fiscal Year: 2016
    ..HCC development is preceded by appearance of foci of altered hepatocytes (FAH), which have been suspected (but never proven) to represent pre-neoplastic lesions...
  16. ALAN D D'ANDREA; Fiscal Year: 2016
    ..We have recently demonstrated that the FAA and FAC proteins bind and form a nuclear complex, although the function of this complex remains unknown...
  17. FANCONI ANEMIA--ROLE OF THE C PROTEIN
    Maureen Hoatlin; Fiscal Year: 2003
    ..function of the FANCC protein, and functions of the proteins encoded by the other more recently cloned FA genes (FANCA and FANCG), the basic defect is still unknown...
  18. Determining the Progenitor-Like Capacity of Liver Cancer Cells in vivo.
    NICOLE K PAULK; Fiscal Year: 2011
    ..liver function in an animal model of metabolic liver disease, the fumarylacetoacetate hydrolase knockout mouse (Fah-/-)...
  19. Evaluation of Novel DExD/H Helicases in Innate Immune Signaling
    Glen N Barber; Fiscal Year: 2013
    ..As second helicase, referred to as Fah-1 (for Fadd-associated helicase), has also been identified, through similar screens using FADD as bait...
  20. In vivo expansion of human hepatocytes in Fah-/-Rag-/-Il2rg-/- mice
    Markus Grompe; Fiscal Year: 2010
    ..Commercialization of our technology will greatly reduce the cost of and accelerate new drug development, thus contributing to public health. ..
  21. Liver Injury and Repopulation by Bone Marrow Stem Cells
    EUGENE SCOTT SWENSON; Fiscal Year: 2010
    ..The FAH knockout mouse is a model of a human liver disease called hereditary tyrosinemia, which affects children and ..
  22. ANDREW ALISTAIR BUTLER; Fiscal Year: 2015
    ..These objectives will be met by assessing FAA and entrainment of circadian rhythms to food in LoxTB MC3R mice crossed with transgenic mouse strains expressing ..
  23. Why we eat when: mechanisms underlying meal entrainment
    Megan Dailey; Fiscal Year: 2012
    ..times of the day as indicated by preprandial increases in both anticipatory activity (food anticipatory activity;FAA) and peptides/hormones (insulin, ghrelin and glucagon-like peptide-1)...
  24. Therapeutic Vaccine for HIV/HPV-associated Oropharyngeal and Tonsillar Malignanci
    FRANK R JONES; Fiscal Year: 2011
    ..data indicate that the AdS [E1-, E2b-] vec*or induces robust CMI responses against tumor associated antigen [fAA), even in the presence of pre- existing AdS immunity...
  25. METABOLISM AND TOXICITY OF HALOGENATED HYDROCARBONS
    MARION ANDERS; Fiscal Year: 2004
    ..Finally, the finding that GSTZ1- 1 also catalyzes the isomerization to MAA to fumarylacetoacetate (FAA) indicates that DCA-induced inactivation of GSTZ 1-1 may decrease the formation of FAA...
  26. FAMILIAL AORTIC ANEURYSM: A MOLECULAR GENETIC ANALYSIS
    Craig Basson; Fiscal Year: 2005
    ..Although monogenic autosomal dominant familial aortic aneurysm (FAA) can occur as one feature of Marfan and Ehlers-Danlos Type IV syndromes, most individuals with FAA do not satisfy ..
  27. INHIBITION OF CARCINOGENESIS BY SELENIUM
    Arthur Cohen; Fiscal Year: 1980
    ..5 ppm) on the incidence of hepatomas in rats fed 2-fluorenylacetamide (FAA). Subsequent experiments will focus on the possible relationship between the antiperoxidase (GPO)...
  28. CREATION OF A MOUSE MODEL OF TYROSINEMIA, TYPE 1
    Markus Grompe; Fiscal Year: 1992
    ..I is an autosomal recessive metabolic disease caused by deficiency of the enzyme fumarylacetoacetate hydrolase (FAH)...
  29. PILOT PERFORMANCE AND ALCOHOL
    Jerome Yesavage; Fiscal Year: 1993
    ..Current Federal Aviation Administration (FAA) regulations permit pilots to fly eight hours after drinking if the blood alcohol concentration (BAC) is less than ..
  30. TYROSINEMIA TYPE I--STRUCTURAL BIOLOGY AND LIVER DISEASE
    David Timm; Fiscal Year: 2000
    ..type I (HT1) is an inherited metabolic disease caused by a deficiency in the enzyme, fumarylacetoacetate hydrolase (FAH)...
  31. Stem Cell-Mediated Liver Repopulation for Murine PKU
    Cary Harding; Fiscal Year: 2006
    ..First, wild type bone marrow will be transplanted into mice that lack both PAH and fumarylacetohydrolase (FAH) activity...
  32. HEMATOPOIESIS AND LEUKEMIC TRANSFORMATION IN FAC MICE
    DAVID CLAPP; Fiscal Year: 2003
    ..The cDNAs of the FA-A, FA-C, and FA-G genes, (FANCA), (FANCC) and (FANCG), respectively, have been identified raising the potential of using gene transfer technology ..
  33. Biochemical Investigations of Fatty Acid Amide Hydrolase
    MICHELE MCKINNEY; Fiscal Year: 2006
    ..demonstrated that fatty acid amide hydrolase (FAAH), an integral membrane enzyme, is the principal regulator of FAA-based signaling events in vivo...
  34. In Situ Simulation to Detect and Prevent Near Misses during Critical Events
    William Riley; Fiscal Year: 2007
    ..Our intervention to improve patient safety is based on an advanced set of event sets and debriefing techniques in use by the Federal Aviation Agency (FAA). [unreadable] [unreadable] [unreadable] [unreadable]
  35. LIVER STEM CELLS AND HUMANIZED MICE: A NEW MODEL FOR HCV
    Eric Lagasse; Fiscal Year: 2002
    ..The same approach will also be applied to fumarylacetoacetate hydrolase (FAH) mice to enrich transplanted human hepatocytes...
  36. Generating a Human Hepatocyte-repopulated Mouse
    Xin Wang; Fiscal Year: 2006
    ..The objective of this application is to create a Rag-2-/- gammac-/- Fah-/- triple-knockout mouse, which will have enhanced capacity of human hepatocyte engraftment compared with previous ..
  37. Christian P Whitman; Fiscal Year: 2016
    ..A fumarylacetoacetate hydrolase (FAH)-like superfamily has now been identified and consists of the decarboxylases in the catechol and hpc pathways and ..
  38. Targeting p27kip1 to Improve Hepatocyte Transplantation
    Erin Ochoa; Fiscal Year: 2009
    ..then shown that donor hepatocytes from p27 knockout mice more actively proliferate and rescue liver failure in the FAH mouse model...
  39. Mechanisms of Amyloid beta-Stimulated ROS in Microglia
    Brandy Wilkinson; Fiscal Year: 2007
    ..proposal is to elucidate mechanisms mediating the microglia pro-inflammatory response to fibrillar beta amyloid (fAa)...
  40. Gene Expression and Discovery in Liver and Gut Stem Cel*
    Gretchen Darlington; Fiscal Year: 2004
    ..markers of mature liver or gut cells, (3) by transplantation to the liver using the fumarylacetoacetate hydrolyze (FAH) deficient mouse, (4) by cDNA microarray analysis, and (5) by a sequence based gene profiling approach...
  41. FANCONI ANEMIA GENE PATHWAY IN RADIATION RESPONSES
    LAWRENCE THOMPSON; Fiscal Year: 2004
    ..The results of these studies will lead to more specific models of the nature of the FA protein "pathway" and its quantitative contributions to multiple biological effects associated with IR-mediated oxidative damage. ..
  42. Microarray Platform for Profiling Cancer Proteases
    Benjamin Cravatt; Fiscal Year: 2008
    ..These proteases may in turn represent valuable new markers and targets for the diagnosis and treatment of cancer. ..
  43. CHEMICAL APPROACHES FOR ACTIVITY BASED PROTEOMICS
    Benjamin Cravatt; Fiscal Year: 2008
    ..unreadable] [unreadable]..
  44. STRUCTURE/FUNCTION STUDIES OF FATTY ACID AMIDE HYDROLASE
    Benjamin Cravatt; Fiscal Year: 2004
    ..abstract_text> ..
  45. Homologous Recombination & Human Cell Radiosensitivity
    LAWRENCE THOMPSON; Fiscal Year: 2009
    ..These integrated studies may lead to a more rational basis for cancer radiotherapy and bring'insights into how HRR prevents the initiation of carcihogenesis by endogenous processes and exogenous agents. ..
  46. Enzymes That Regulate Fatty Acid Amide Function In Vivo
    Benjamin Cravatt; Fiscal Year: 2009
    ..A prototype FAA anandamide serves as an endogenous ligand for the central cannabinoid (CB1) receptor...
  47. GENETIC ANALYSIS OF NUCLEOTIDE EXCISION REPAIR
    LAWRENCE THOMPSON; Fiscal Year: 2002
    ..These mechanistic insights will guide future structural studies on ERCC2. ..
  48. Drug Abuse Related Polymorphism in Fatty Acid Amide Hydrolase
    Benjamin Cravatt; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  49. Clinical Applications of a Novel Fanconi Anemia Assay
    Akiko Shimamura; Fiscal Year: 2007
    ..abstract_text> ..
  50. MRI and Decline of Aging Aviator Performance
    Joy Taylor; Fiscal Year: 2007
    ..We propose to enhance our set of predictors by the addition of highly quantified MR volumetric measures to better explain in whom and how functional decline OCCU rs. ..
  51. Colon and liver anticarcinogenicity by acetaminophen
    Gary Williams; Fiscal Year: 2004
    ..In addition, these experiments will provide support for the epidemiological observations of reduced cancer risk associated with the use of APAP. ..
  52. Mutagenesis of the Mammalian Imprinting Process(es)
    Eugene Rinchik; Fiscal Year: 2002
    ..abstract_text> ..
  53. Isolation of Murine Pancreatic Liver Stem Cells
    Markus Grompe; Fiscal Year: 2004
    ..Monoclonal antibodies useful for FACS sorting of pancreatic cells will be generated. We will apply cell-sorting methods to enrich pancreatic liver stem cells. ..
  54. Cell Therapy by In Vivo Fusion
    Markus Grompe; Fiscal Year: 2007
    ..restore abnormal liver function in an animal model of metabolic liver disease, the fumarylacetoacetate hydrolase (Fah) knockout mouse...
  55. daf-12 Target Genes during C. elegans aging
    Alfred Fisher; Fiscal Year: 2005
    ..abstract_text> ..
  56. Timing: Pharmacology and Conditioning
    Peter Balsam; Fiscal Year: 2005
    ..Consequently, a better understanding of conditioning processes that accompany drug taking can also lead to more effective interventions. ..
  57. Interdisciplinary Training-Alcohol, Injury, & Violence
    Susan Baker; Fiscal Year: 2005
    ..The program aims to develop creative and independent investigators, well prepared to contribute to amelioration of alcohol-related injury. ..
  58. Fine-mapping of 8q24 and Loci Identified in Genome-Wide Studies for Breast & Colo
    CHRISTOPHER ALAN HAIMAN; Fiscal Year: 2010
    ..We expect findings from this work to guide the development of future preventive, early detection and prognostic strategies ..
  59. Circadian organization in the aged rodent
    Alec Davidson; Fiscal Year: 2004
    ....
  60. FASEB Conference: Mechanisms of Liver Growth and Disease
    Markus Grompe; Fiscal Year: 2004
    ..abstract_text> ..
  61. Olfactory Development: Cell Proliferation and Maturation
    CHARLES DERBY; Fiscal Year: 2007
    ..abstract_text> ..
  62. Mechanisms of In Vivo Cell Fusion
    Andrew Duncan; Fiscal Year: 2008
    ..restore abnormal liver function in an animal model of metabolic liver disease, the fumarylacetoacetate hydrolase (Fah) knockout mouse...
  63. A Comprehensive Analysis of Genetic Variation in DNA Repair Genes in Relation to
    Christopher Haiman; Fiscal Year: 2007
    ..The ability to accurately identify high-risk sub-groups may have profound implications on endometrial cancer prevention as well as advance research aimed at targeted drug development. [unreadable] [unreadable] [unreadable]..
  64. Nutritional manipulation of circadian rhythms and cancer
    Alec Davidson; Fiscal Year: 2007
    ..The experiments will form the basis for future study of circadian and nutritional influences on development of this and other cancers. [unreadable] [unreadable] [unreadable]..
  65. RETROVIRAL TRANSFER OF ANKYRIN FOR SPHEROCYTOSIS
    Pamela Becker; Fiscal Year: 2002
    ..abstract_text> ..
  66. GENETIC ANALYSIS OF ENZYME PROCESSING AND LOCALIZATION
    Elizabeth Jones; Fiscal Year: 2002
    ..Methods will include targeted mutagenesis, suppressor selection and analysis, subcellular fractionation, biochemistry, and immunofluorescence. ..