Porphobilinogen deaminase over-expression in hepatocytes, but not in erythrocytes, prevents accumulation of toxic porphyrin precursors in a mouse model of acute intermittent porphyria

Journal of Hepatology

Volumn 52, Issue 3, 2010, Pages 417-424

  Unzu, Carmen ^a   Sampedro, Ana ^a   Mauleón, Itsaso ^a   Vanrell, Lucía ^a   Dubrot, Juan ^a   de Salamanca, Rafael Enríquez ^b   González Aseguinolaza, Gloria ^a   Melero, Ignacio ^a   Prieto, Jesús ^a   Fontanellas, Antonio ^a  

^a UNIVERSITY OF NAVARRA   (Spain)

^b HOSPITAL UNIVERSITARIO 12 DE OCTUBRE   (Spain)

Author keywords

Acute attack; Acute intermittent porphyria; Bone marrow transplant; Liver gene transfer; Motor coordination; Porphyrin precursor excretion

Indexed keywords

LUCIFERASE; PORPHOBILINOGEN DEAMINASE; PORPHYRIN;

ACUTE INTERMITTENT PORPHYRIA; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BONE MARROW TRANSPLANTATION; CELL ACTIVITY; CONTROLLED STUDY; DONOR; ENZYME ACTIVITY; ERYTHROCYTE; ERYTHROID CELL; FEMALE; GENE THERAPY; GENE TRANSFER; HEMATOPOIETIC CELL; HYDRODYNAMICS; LIVER CELL; MOTOR COORDINATION; MOTOR DYSFUNCTION; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; PLASMID; PRIORITY JOURNAL; PROTEIN EXPRESSION; URINARY EXCRETION; WILD TYPE;

AMINOLEVULINIC ACID; ANIMALS; BONE MARROW TRANSPLANTATION; DISEASE MODELS, ANIMAL; ERYTHROCYTES; FEMALE; GENE TRANSFER TECHNIQUES; HEPATOCYTES; HUMANS; HYDROXYMETHYLBILANE SYNTHASE; MICE; MICE, INBRED C57BL; PLASMIDS; PORPHOBILINOGEN; PORPHYRIA, ACUTE INTERMITTENT; PORPHYRINS;

EID: 77249122441     PISSN: 01688278     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jhep.2009.09.003     Document Type: Article

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