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

UV-sensitive syndrome (UV(S)S) and Cockayne syndrome (CS) are human disorders caused by CSA or CSB gene mutations; both conditions cause defective transcription-coupled repair and photosensitivity. Patients with CS also display neurological and developmental abnormalities and dramatic premature agin...

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Veröffentlicht in:Proc Natl Acad Sci U S A
Hauptverfasser: Chatre, Laurent, Biard, Denis S. F., Sarasin, Alain, Ricchetti, Miria
Format: Artigo
Sprache:Inglês
Veröffentlicht: National Academy of Sciences 2015
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Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4460464/
https://ncbi.nlm.nih.gov/pubmed/26038566
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1422264112
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