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. 2020 Dec 6;9(12):2620. doi: 10.3390/cells9122620

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© 2020 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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HIGD1A interacts with CIV and enhances its activity. Following a proposed model [31,70], overexpressed HIGD1A interacts with cytochrome c and CIV and produces a structural change in the CIV heme a active center, acting as a positive modulator of the enzyme. The figure was generated with PyMOL and Adobe Photoshop using the structure of the CI-CIII₂-CIV respirasome from ovine (Ovis aries) heart mitochondria [81](PDB 5J4Z). IMS, intermembrane space. IMM, inner mitochondrial membrane. CI, complex I. CIII₂, complex III dimer. CIV, complex IV.