## Abstract Defects in NADH:ubiquinone oxidoreductase (complex I), the largest complex of the mitochondrial respiratory chain, account for most cases of respiratory chain deficiency in human. Complex I contains at least 45 subunits, 7 of which are encoded by mitochondrial DNA (mtDNA). Here we repor
✦ LIBER ✦
Progressive cavitating leukoencephalopathy associated with respiratory chain complex I deficiency and a novel mutation inNDUFS1
✍ Scribed by Mariana Ferreira; Alessandra Torraco; Teresa Rizza; Fabiana Fattori; Maria Chiara Meschini; Cinzia Castana; Nancy E. Go; Frank E. Nargang; Margarida Duarte; Fiorella Piemonte; Carlo Dionisi-Vici; Arnaldo Videira; Laura Vilarinho; Filippo M. Santorelli; Rosalba Carrozzo; Enrico Bertini
- Publisher
- Springer
- Year
- 2011
- Tongue
- English
- Weight
- 509 KB
- Volume
- 12
- Category
- Article
- ISSN
- 1364-6745
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## Abstract We have used HeLa cells without mitochondrial DNA (ρ^0^‐cells) and transient ρ^0^‐phenocopies, obtained from wild‐type cells by short‐term treatment with ethidium bromide, to analyze how the absence of a functional mitochondrial respiratory chain slows down proliferation. We ruled out a