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2005 Congress Abstracts

Abstracts 2005

5-AMINOLEVULINATE SYNTHASE SCAFFOLD AND CONVERSION INTO A MORE ACTIVE ENZYME

J.-S. Zhang, G. A. Hunter and G. C. Ferreira

Department of Biochemistry and Molecular Biology, College of Medicine and H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, Florida 33612, USA.

5-AMINOLEVULINIC ACID ALTERS THE ANTIOXIDANT ENZYME SYSTEM IN MICE BRAIN

Jorge Rodriguez, Alcira Batlle & Ana Maria Buzaleh

Centro de Investigaciones sobre Porfirinas y Porfirias (CIPYP) - CONICET. University of Buenos Aires. Argentina

A CASE OF ALAD PORPHYRIA IN NORTH AMERICA: RESPONSE TO HEMIN THERAPY

1Chul Lee, 1Karl E. Anderson, 2Shigeru Sassa and 3Reiko Akagi

1The University of Texas Medical Branch, Galveston, Texas, USA; 2Rockefeller University, New York, USA; 3Okayama Prefectural University, Okayama-ken, Japan

A TRUNCATED FORM OF HEME OXYGENASE-1 IS FOUND IN THE NUCLEUS OF CULTURED CELLS IN RESPONSE TO INCREASES IN INTRACELLULAR HEME

Guang Yang, Ph.D.*, Qing Lin, Ph.D.*, Yi-Hao Weng, M.D.#. Kimberly Rish, M.S.**, Ann Smith, Ph.D. ** and Phyllis A. Dennery, M.D.

*†#.Children's Hospital of Philadelphia*, Department of Pediatrics, University of Pennsylvania†, Philadelphia, Pennsylvania; School of Biological Sciences, University of Missouri-Kansas City**, Kansas City, Missouri; and Department of Pediatrics, Stanford University#, California, U.S.A.

ACTIVE SITE OF HUMAN FERROCHELATASE

H.A. Dailey, C.-K. Wu, W.N. Missaoui, P. Horanyi, B.-C. Wang, J. Rose, and T.A. Dailey

Biomedical and Health Sciences Institute, Department of Microbiology, and Department of Biochemistry and Molecular Biology, University of Georgia, Athens, GA 30602

ADENOVIRAL-MEDIATED EXPRESSION OF PORPHOBILINOGEN DEAMINASE IN LIVER RESTORES THE METABOLIC DEFECT IN A MOUSE MODEL OF ACUTE INTERMITTENT PORPHYRIA

Johansson A1,*, Nowak G2, Möller C3, Blomberg P4, Harper P1

1Porphyria Centre Sweden, Division of Clinical Chemistry, 2Division of Transplantation Surgery, Department of Laboratory Medicine, 4Gene Therapy Center, Karolinska University Hospital, Huddinge, 141 86 Stockholm, Sweden, 3HemeBiotech A/S, Lidingö, Sweden, *Annika.Johansson@mssm.edu

ALA-PDT SENSITIVITY OF CELL LINES RESISTANT TO NITRIC OXIDE CYTOTOXICITY

Gabriela Divenosa, Adriana Casas, Christian Perotti, Haydee Fukuda & Alcira Batlle

Centro de Investigaciones dsbre Porfirinas y Porfirias (CIPYP), CONICET and University of Buenos Aires

In an attempt to elucidate interactions between aminolevulinic acid-based photodynamic therapy (ALA-PDT) and nitric oxide (NO) cytotoxicity, we employed a murine mammary adenocarcinoma cell line LM3 and the NO resistant variant LM3-SNP.

ANCESTRAL FOUNDER OF MUTATION R116W IN THE PORPHOBILINOGEN DEAMINASE GENE AMONG DUTCH ACUTE INTERMITTENT PORPHYRIA PATIENTS

F.W.M. de Rooij, F.G. Kavelaars, H. Koole-Lesuis, J.H.P. Wilson

Dept. of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Introduction:  Acute intermittent porphyria (AIP), the most common acute hepatic porphyria, is an autosomal dominant inborn disorder of heme biosynthesis caused by mutations in the porphobilinogen deaminase (PBGD) gene. The prevalence of AIP in Europe is estimated as 1/10.000-1/20.000.

ATYPICAL RED CELL INCLUSIONS IN A PATIENT WITH CONGENITAL ERYTHROPOIETIC PORPHYRIA

A Merino, M Rozman*, C Herrero**, J To-Figueras, A Ordinas

Servicio de Hemoterapia-Hemostasia. Core Lab. *Unidad de Hematopatología. CDB.**ICMD. Hospital Clínic. IDIBAPS. Universidad de Barcelona

We show the morphological finding of some atypical inclusions in mature red cells and their precursors in a 28 years-old patient with congenital erythropoietic porphyria.

AUTOSOMAL RECESSIVE ERYTHROPOIETIC PROTOPORPHYRIA WITHOUT IVS3-48C IN TWO BROTHERS WITH LIVER DYSFUNCTION IN EARLY CHILDHOOD

F.W.M. de Rooij, K. Munte, A. Edixhoven, R. Koole, J.H.P. Wilson

Erasmus MC, Rotterdam, The Netherlands

BIOCHEMICAL AND GENETIC CHARACTERIZATION OF FOUR CASES OF HEREDITARY COPROPORPHYRIA IN SPAIN

Carmen Herrero , Jordi To-Figueras , Celia Badenas , Maria T Enríquez (*), Sonia Segura , Concepción Alvarez (**), Montserrat Milà , Marius Lecha

Porphyria , Dermatology and Genetics Unit. Hospital Clínic. University of Barcelona. Villarroel 170. 08036 Barcelona. Spain; (*): Pediatrics Service. Hospital Axarquia. Malaga; (**): Hospital Marques de Valdecilla. Santander. Spain

CHARACTERISATION OF ERYTHROPOIETIC PROTOPORPHYRIA IN SOUTH AFRICA

Michelle Parker , Anne V. Corrigall and Peter N. Meissner

Lennox Eales Porphyria Laboratories, MRC/UCT Liver Research Centre, Department of Medicine, University of Cape Town Medical School, Observatory 7925, South Africa

CHARACTERISATION OF MITOCHONDRIAL TARGETING OF WILD TYPE AND MUTANT (L88Q) HUMAN COPROPORPHYRINOGEN OXIDASE

Van Der Merwe D, Roberts AG, Badminton MN

Departments of Medical Biochemistry and Immunology, University Hospital of Wales and School of Medicine, Cardiff University, Cardiff

CLINICAL, BIOCHEMICAL AND PATHOHISTOLOGICAL ASPECTS IN 12 PATIENTS WITH ACUTE PORPHYRIA AND HEPATOCELLULAR CANCER

Sardh E#, Harper P*, Andersson DEH#

#Department of Internal medicine, Stockholm Söder Hospital and *Porphyria Centre Sweden, Karolinska University Hospital Huddinge, Stockholm, Sweden. Eliane.Sardh@sos.ki.se

The aim of the study was to retrospectively study 10 women and 2 men with acute porphyria (10 AIP, 1 HCP, 1 VP) who had developed hepatocellular cancer (HCC).

CONCURRENT UPREGULATION OF HAEM OXYGENASE 1 AND CYTOCHROME P450 2A5: POSSIBLE ROLE OF Nrf2 AND hnRNP A1

Michael R. Moore1, Aedah A. Bakar1, Matti A Lang2

1EnTox, The University of Queensland, Australia, 2Department of Biochemistry, Uppsala University, Sweden

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