Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10795924 | Biochimica et Biophysica Acta (BBA) - Bioenergetics | 2012 | 7 Pages |
Abstract
⺠ND1 subunit is adjacent to the ubiquinone binding site of complex I. ⺠Involvement of E. coli NuoH-E36, the homolog of ND1-E24, in quinone binding is shown. ⺠ND1-E24 lies in the ubiquinone binding domain delineated by PSST and 49 kDa subunits. ⺠NUOH-E36 mutation effects corroborate the m.3376G > A pathogenicity in LHON/MELAS. ⺠m.3376G > A combines enzymic consequences of separate LHON and MELAS mutations.
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Authors
Jukka Pätsi, Pilvi Maliniemi, Salla Pakanen, Reetta Hinttala, Johanna Uusimaa, Kari Majamaa, Thomas Nyström, Marko Kervinen, Ilmo E. Hassinen,