NDUFS8

NDUFS8

Target · Mitochondrial enzyme subunit

NDUFS8 NADH:ubiquinone oxidoreductase core subunit S8 · TYKY

NDUFS8 (NADH:ubiquinone oxidoreductase core subunit S8, also known as TYKY and CI-23k) is a core subunit of mitochondrial Complex I, the NADH dehydrogenase of the respiratory chain. It is essential for the catalytic activity and assembly of the complex. Browse Assay Genie's current range of NDUFS8 research reagents below, or read on for the biology behind the target.

Mitochondrial inner membraneComplex I core subunitLinked to Leigh syndrome
210 aa
Protein length
~24 kDa
Molecular weight
11q13.2
Gene locus
EC 7.1.1.2
Enzyme class
Target information

What is NDUFS8 (NADH:ubiquinone oxidoreductase core subunit S8)?

NDUFS8 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, known as Complex I. This multimeric enzyme catalyses electron transfer from NADH through the respiratory chain, using ubiquinone as the electron acceptor. According to the RefSeq summary, Complex I is responsible for NADH oxidation, ubiquinone reduction and the ejection of protons from mitochondria. The NDUFS8 protein is essential for both the catalytic activity and the assembly of the complex.

The NDUFS8 protein is involved in binding two of the six to eight iron-sulfur clusters of Complex I and is therefore required for the electron transfer process. It localises to the mitochondrion inner membrane and belongs to the complex I 23 kDa subunit family, which is reflected in the alias CI-23k. The NDUFS8 gene is classed by HGNC among the NADH:ubiquinone oxidoreductase core subunits, and it is also listed as a microRNA protein coding host gene. The mouse ortholog is Ndufs8.

The gene was previously named NADH dehydrogenase (ubiquinone) Fe-S protein 8 (23kD), or NADH-coenzyme Q reductase, and TYKY is another recorded alias. Mutations in the NDUFS8 gene have been associated with Leigh syndrome, which makes the protein a point of interest for researchers studying Complex I function and mitochondrial disease.

Respiratory chain biology

NDUFS8 is a core Complex I subunit that supports electron transfer from NADH to ubiquinone, so it is relevant to studies of oxidative phosphorylation.

Complex I assembly

The protein is essential for the assembly of Complex I, making it useful for work on how the multisubunit enzyme is built.

Mitochondrial disease research

Mutations in NDUFS8 have been associated with Leigh syndrome, linking the protein to studies of inherited mitochondrial disorders.

Iron-sulfur cluster function

NDUFS8 is involved in binding two of the iron-sulfur clusters of Complex I, which are required for electron transfer.

Background

NDUFS8 frequently asked questions

What does NDUFS8 do?

NDUFS8 is a core subunit of mitochondrial Complex I, which catalyses electron transfer from NADH to ubiquinone. It is essential for the catalytic activity and assembly of the complex, and it is involved in binding two of Complex I's iron-sulfur clusters, which are needed for electron transfer.

What other names does NDUFS8 go by?

NDUFS8 is also called TYKY and CI-23k. The full HGNC name is NADH:ubiquinone oxidoreductase core subunit S8, and older names include NADH dehydrogenase (ubiquinone) Fe-S protein 8 (23kD) and NADH-coenzyme Q reductase. UniProt lists it as an iron-sulfur protein 8, mitochondrial.

Is NDUFS8 linked to disease, and is it conserved in mouse?

Mutations in the NDUFS8 gene have been associated with Leigh syndrome. The mouse ortholog is Ndufs8, so mouse models can be used to study the protein. Check the datasheet of any product for its validated reactivity.

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Related targets

Other Complex I iron-sulfur protein subunits and mitochondrial respiratory chain targets with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt O00217; product specifications from Assay Genie datasheets.

NDUFS8 Product listing