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Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond.
- Source :
- Mitochondrion; 57; 65; 1567-7249; 1; 12; ~Mitochondrion~57~65~~~1567-7249~1~12~~
- Publication Year :
- 2012
-
Abstract
- 1 januari 2012<br />Item does not contain fulltext<br />Complex I (CI) represents a major entry point of electrons in the mitochondrial electron transport chain (ETC). It consists of 45 different subunits, encoded by the mitochondrial (mtDNA) and nuclear DNA (nDNA). In humans, mutations in nDNA-encoded subunits cause severe neurodegenerative disorders like Leigh Syndrome with onset in early childhood. The pathophysiological mechanism of these disorders is still poorly understood. Here we summarize the current knowledge concerning the consequences of nDNA-encoded CI mutations in patient-derived cells, present mouse models for human CI deficiency, and discuss potential treatment strategies for CI deficiency.
Details
- Database :
- OAIster
- Journal :
- Mitochondrion; 57; 65; 1567-7249; 1; 12; ~Mitochondrion~57~65~~~1567-7249~1~12~~
- Publication Type :
- Electronic Resource
- Accession number :
- edsoai.on1284165900
- Document Type :
- Electronic Resource