Reversal of mitochondrial defects with CSB-dependent serine protease inhibitors in patient cells of the progeroid Cockayne syndrome

Proceedings of the National Academy of Sciences of the United States of America

Volumn 112, Issue 22, 2015, Pages E2910-E2919

  Chatre, Laurent ^a,b   Biard, Denis S F ^c   Sarasin, Alain ^d,e   Ricchetti, Miria ^a,b  

^a INSTITUT PASTEUR   (France)

^b Team Stability of Nuclear and Mitochondrial DNA   (France)

^c CEA DSV iMETI SEPIA   (France)

^d Gustave Roussy Comprehensive Cancer Institute   (France)

^e INSTITUT GUSTAVE ROUSSY   (France)

Author keywords

Cockayne syndrome; DNA polymerase gamma; mtSSB; Premature ageing; Serine protease

Indexed keywords

DNA DIRECTED DNA POLYMERASE GAMMA; HTRA3 SERINE PROTEASE; MITOCHONDRIAL DNA; PEROXYNITRITE; PROTEIN POLG1; REACTIVE OXYGEN METABOLITE; SCAVENGER; SERINE PROTEINASE; SERINE PROTEINASE INHIBITOR; UNCLASSIFIED DRUG; DNA DIRECTED DNA POLYMERASE; DNA HELICASE; DNA LIGASE; ERCC6 PROTEIN, HUMAN; PEROXYNITROUS ACID;

ARTICLE; BIOACCUMULATION; COCKAYNE SYNDROME; CONTROLLED STUDY; CSB GENE; DISORDERS OF MITOCHONDRIAL FUNCTIONS; GENE; GENE SILENCING; HUMAN; HUMAN CELL; NITROSATIVE STRESS; OXIDATION REDUCTION STATE; OXIDATIVE PHOSPHORYLATION; PHENOTYPE; PHOTOSENSITIVITY DISORDER; PREMATURE AGING; PRIORITY JOURNAL; REDOX STRESS; SKIN FIBROBLAST; ULTRAVIOLET SENSITIVE SYNDROME; CELL CULTURE; FLUORESCENT ANTIBODY TECHNIQUE; HELA CELL LINE; METABOLISM; MITOCHONDRIAL DISEASES; PATHOLOGY; PROGERIA; REAL TIME POLYMERASE CHAIN REACTION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; WESTERN BLOTTING;

BLOTTING, WESTERN; CELLS, CULTURED; COCKAYNE SYNDROME; DNA HELICASES; DNA REPAIR ENZYMES; DNA, MITOCHONDRIAL; DNA-DIRECTED DNA POLYMERASE; FLUORESCENT ANTIBODY TECHNIQUE; HELA CELLS; HUMANS; MITOCHONDRIAL DISEASES; PEROXYNITROUS ACID; PROGERIA; REACTIVE OXYGEN SPECIES; REAL-TIME POLYMERASE CHAIN REACTION; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; SERINE PROTEINASE INHIBITORS;

EID: 84930960039     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1422264112     Document Type: Article

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