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. 2020 Sep 25;10:15788. doi: 10.1038/s41598-020-72884-w

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Amino acid sequences, predicted MPP cleavage sites, a Glu-C cleavage site, and N-terminal tryptic peptides. (A) Mouse mature frataxin (78-207) is formed by MPP-mediated cleavage of mouse full-length frataxin at G⁴⁰-L⁴¹ followed by cleavage of the resulting intermediate frataxin (41-207) at N⁷⁷-L⁷⁸. Trypsin digestion of mature mouse frataxin results in formation of the N-terminal peptide L⁷⁸GTLDNPSSLDETAYER⁹⁴; whereas, digestion with the endopeptidase Glu-C results in formation of the N-terminal peptide L⁷⁸GTLDNPSSLDETAYE⁹³. (B) Human mature frataxin is formed by MPP-mediated cleavage of human full-length frataxin at G⁴¹-L⁴² followed by cleavage of the resulting intermediate frataxin (42-210) at K⁸⁰-S⁸¹. Trypsin digestion of mature human frataxin results in formation of the N-terminal peptide S⁸¹GTLGHPGSLDE⁸⁹.