Accessory Subunits of the Matrix Arm of Mitochondrial Complex I with a Focus on Subunit NDUFS4 and Its Role in Complex I Function and Assembly.
作者： Volker Zickermann , Flora Kahlhöfer , Max Gansen
DOI: 10.3390/LIFE11050455
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摘要: NADH:ubiquinone-oxidoreductase (complex I) is the largest membrane protein complex of respiratory chain. Complex I couples electron transfer to vectorial proton translocation across inner mitochondrial membrane. The L shaped structure divided into a arm and matrix arm. Fourteen central subunits are conserved throughout species, while some 30 accessory typically found in eukaryotes. dysfunction associated with mutations nuclear genome, resulting broad spectrum neuromuscular neurodegenerative diseases. Accessory subunit NDUFS4 hot spot for causing Leigh or Leigh-like syndrome. In this review, we focus on discuss recent reports function its interplay NDUFS6, NDUFA12, assembly factor NDUFAF2 assembly.
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