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9.A.62 The AAA-ATPase, Bcs1 (Bcs1) Family

The mitochondrial AAA-ATPase, Bcs1, mediates topogenesis of the Rieske protein, Rip1, a component of respiratory chains in bacteria, mitochondria, and chloroplasts (Wagener et al., 2011). The oligomeric AAA-ATPase Bcs1 is involved in export of the folded Fe-S domain of Rip1 across the inner membrane and insertion of its transmembrane segment into an assembly intermediate of the cytochrome bc(1) complex. Structural elements in Rip1 are required for recognition and export by as well as ATP-dependent lateral release from the AAA-ATPase. In bacteria and chloroplasts Rip1 uses the Tat machinery for topogenesis; however, mitochondria have lost this machinery during evolution and a member of the AAA-ATPase family has taken over its function (Wagener et al., 2011).  Mutations in Bcs1 lead to different properties of the protein and different disease-related symptoms (Wagener and Neupert 2012).

This family belongs to the: AAA-ATPase Superfamily.

References associated with 9.A.62 family:

Wagener, N. and W. Neupert. (2012). Bcs1, a AAA protein of the mitochondria with a role in the biogenesis of the respiratory chain. J Struct Biol 179: 121-125. 22575765
Wagener, N., M. Ackermann, S. Funes, and W. Neupert. (2011). A pathway of protein translocation in mitochondria mediated by the AAA-ATPase Bcs1. Mol. Cell 44: 191-202. 22017868