NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7)

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NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7) also knowns as NADH-ubiquinone oxidoreductase 20 kDa subunit, Complex I-20kD (CI-20kD), or PSST subunit is an enzyme that in humans is encoded by the NDUFS7 gene. The PSST subunit encoded by NDUSF7 gene is one of more than 40 subunits involved in electron transfer from NADH to ubiquinone. This complex plays a role in the transfer of electrons from NADH to respiratory chain, and ubiquinone is considered to be the direct electron acceptor of the enzyme. Specifically, it is considered that the electron transfer between the iron-sulfur cluster N2 and the ubiquinone is directly coupled between the PSST subunit and the ubiquinone-bound ND1. The mutation of this gene causes Leigh syndrome due to the lack of mitochondrial complex I, which is a serious neurological disease that leads to bilaterally symmetrical necrotic lesions in subcortical brain regions.

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