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The Assembly of Human Complex I

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A Structural Perspective on Respiratory Complex I

Abstract

Human mitochondrial complex I is one of the biggest membrane bound enzymes found in man. Its assembly is a complicated process and requires the combination of 45 subunits, encoded by the nuclear and mitochondrial genomes. It has become clear that the assembly of complex I takes place in different steps in which some parts are assembled separately and connected to form a functional enzyme. Remarkably this stepwise process follows the assembly of the distinct functional and evolutionary conserved modules, the hydrogenase part, the membrane transporter part and the NADH dehydrogenase part. Assembly of these parts and further combination of these modules requires the help of specialized proteins called chaperones. A number of these chaperones have been identified and they play a role in the different stages of the assembly. Although the precise molecular mechanisms concerning complex I assembly are still unknown, the overall picture of this process is getting shape as discussed in this chapter.

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Authors and Affiliations

  1. Department of Pediatrics, Nijmegen Center for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, 9101, 6500 HB, Nijmegen, The Netherlands

    Jessica Nouws, Maria Antonietta Calvaruso & Leo Nijtmans

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  1. Jessica Nouws
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  2. Maria Antonietta Calvaruso
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  3. Leo Nijtmans
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Correspondence to Leo Nijtmans .

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  1. , Mitochondrial Biology Unit, Medical Research Council, Hills road, Cambridge, CB2 0XY, Cambridgeshire, United Kingdom

    Leonid Sazanov

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Nouws, J., Calvaruso, M.A., Nijtmans, L. (2012). The Assembly of Human Complex I. In: Sazanov, L. (eds) A Structural Perspective on Respiratory Complex I. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4138-6_10

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