The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes

Biochimica et Biophysica Acta - Molecular Cell Research

Volumn 1783, Issue 10, 2008, Pages 1707-1717

  Bonnet, Crystel ^a   Augustin, Sébastien ^a   Ellouze, Sami ^a   Bénit, Paule ^c   Bouaita, Aicha ^a   Rustin, Pierre ^c   Sahel, José Alain ^a,b   Corral Debrinski, Marisol ^a  

^a INSTITUT DE LA VISION   (France)

^b CENTRE HOSPITALIER NATIONAL D OPHTALMOLOGIE DES QUINZE VINGTS   (France)

^c HÔPITAL ROBERT DEBRÉ   (France)

Author keywords

Complex I and V activities; LHON; Nuclearly encoded ND1 and ND4 genes; Oxidative phosphorylation

Indexed keywords

ADENOSINE TRIPHOSPHATE; GALACTOSE; GLUCOSE; HYBRID PROTEIN; MESSENGER RNA; MITOCHONDRIAL DNA; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE);

ADULT; ARTICLE; BIOCHEMICAL COMPOSITION; CONTROLLED STUDY; ENZYME ACTIVITY; GENE; GENE EXPRESSION; GENE MUTATION; HUMAN; HUMAN CELL; IMMUNOCYTOCHEMISTRY; LEBER HEREDITARY OPTIC NEUROPATHY; MALE; MITOCHONDRION; NUCLEARLY ENCODED 1 GENE; NUCLEARLY ENCODED 4 GENE; OXIDATIVE PHOSPHORYLATION; PRESCHOOL CHILD; PRIORITY JOURNAL; RESPIRATORY CHAIN; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; RNA TRANSPORT; SKIN FIBROBLAST; STATISTICAL SIGNIFICANCE; WESTERN BLOTTING;

ADENOSINE TRIPHOSPHATE; ADOLESCENT; ADULT; CELLS, CULTURED; CULTURE MEDIA; ELECTRON TRANSPORT; FIBROBLASTS; GALACTOSE; GENE EXPRESSION REGULATION, ENZYMOLOGIC; HUMANS; MALE; MUTATION; NADH DEHYDROGENASE; PROTEIN BINDING; RNA, MESSENGER; TRANSGENES;

EID: 50849085156     PISSN: 01674889     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbamcr.2008.04.018     Document Type: Article

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