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. Author manuscript; available in PMC: 2021 Sep 30.
Published in final edited form as: Nat Struct Mol Biol. 2020 Feb 10;27(2):202–209. doi: 10.1038/s41594-020-0373-0

Extended Data Fig. 10 |. Pathogenic mutations of BCS1L.

Extended Data Fig. 10 |

a, Pathogenic mutations of BCS1L and their locations in the Bcs1 structure. b–d, Mapping of the mutations on ATPγS-bound and apo mBcs1 in two orthogonal orientations: top view (top) and side view (bottom). TM, Bcs1-specific and AAA regions are in black, blue and magenta ribbons, respectively. Mutations are showed in spheres. Mutations found in (b), Björnstad syndrome; (c) GRACILE syndrome and (d) Complex III deficiency.