Respiratory complex I

<p>Respiratory complex I, <a href="/facts/Enzyme_Commission_number/Y5GNLRKX">EC</a> <a href="https://enzyme.expasy.org/EC/7.1.1.2">7.1.1.2</a> (also known as NADH:ubiquinone oxidoreductase, Type I NADH dehydrogenase and mitochondrial complex I) is the first large <a href="/facts/Protein_complex/GW11Ee34">protein complex</a> of the <a href="/facts/Electron_transport_chain/QjcIkgeR">respiratory chains</a> of many organisms from bacteria to humans. It catalyzes the transfer of <a href="/facts/Electron/L0KBo5cW">electrons</a> from <a href="/facts/NADH/BHdLW925">NADH</a> to <a href="/facts/Coenzyme_Q10/xoeh3R3e">coenzyme Q10</a> (CoQ10) and translocates protons across the inner <a href="/facts/Mitochondria/ErHqrdJ1">mitochondrial</a> membrane in eukaryotes or the plasma membrane of bacteria.
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<p>This enzyme is essential for the normal functioning of cells, and mutations in its subunits lead to a wide range of inherited neuromuscular and metabolic disorders. Defects in this enzyme are responsible for the development of several pathological processes such as <a href="/facts/Reperfusion_injury/3MWNux98">ischemia/reperfusion</a> damage (<a href="/facts/Stroke/DwpjdzY0">stroke</a> and <a href="/facts/Myocardial_infarction/xoGw0qcG">cardiac infarction</a>), Parkinson's disease and others.
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Respiratory complex I open-in-new

Respiratory complex I