NDUFS2

NDUFS2

Target · Mitochondrial enzyme subunit

NDUFS2 NADH:ubiquinone oxidoreductase core subunit S2 · NADH dehydrogenase [ubiquinone] iron-sulfur protein 2

NDUFS2 (NADH:ubiquinone oxidoreductase core subunit S2, also known as CI-49) is a core subunit of mitochondrial respiratory chain Complex I. It supports electron transfer from NADH to ubiquinone and is needed for Complex I assembly. Browse Assay Genie's current range of NDUFS2 research reagents below, or read on for the biology behind the target.

Mitochondrial inner membraneComplex I core subunitOxygen-sensing role
463 aa
Protein length
~53 kDa
Molecular weight
1q23.3
Gene locus
EC 7.1.1.2
Enzyme class
Target information

What is NDUFS2 (NADH:ubiquinone oxidoreductase core subunit S2)?

NDUFS2 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, also called Complex I. Complex I catalyses electron transfer from NADH through the respiratory chain, using ubiquinone as the electron acceptor, and the reaction is coupled to proton ejection out of the mitochondria. NDUFS2 is essential for the catalytic activity of Complex I and for its assembly. The protein is also redox-sensitive and is described as a critical component of the oxygen-sensing pathway in the pulmonary vasculature, where it contributes to acute pulmonary oxygen sensing and hypoxic pulmonary vasoconstriction. It also plays an important role in carotid body sensing of hypoxia.

The NDUFS2 gene encodes a protein of the complex I 49 kDa subunit family, which accounts for the earlier name CI-49 and the previous designation NADH dehydrogenase (ubiquinone) Fe-S protein 2 (49kD). The protein is located in the mitochondrial inner membrane. Mammalian Complex I is composed of at least 43 different subunits, 7 of them encoded by the mitochondrial genome and the remainder by nuclear genes. NDUFS2, a nuclear gene product, belongs to the iron-sulfur protein fraction of the complex, which comprises 7 subunits. Alternatively spliced transcript variants encoding different isoforms have been reported. Mouse and rat both have an orthologous Ndufs2 gene.

Mutations in the NDUFS2 gene are associated with mitochondrial complex I deficiency. UniProt additionally reports that NDUFS2 is essential for the proliferation and differentiation of glia-like neural stem and progenitor cells and for the later maturation of oligodendrocytes or neurons. These roles make NDUFS2 a useful target for studies of mitochondrial bioenergetics, oxygen sensing and neural development.

Complex I biology

As a core catalytic subunit, NDUFS2 is central to studies of NADH oxidation, ubiquinone reduction and proton ejection by Complex I.

Mitochondrial disease research

Mutations in NDUFS2 are associated with mitochondrial complex I deficiency, linking the gene to studies of respiratory chain dysfunction.

Oxygen sensing and hypoxia

NDUFS2 is a redox-sensitive component of pulmonary oxygen sensing, hypoxic pulmonary vasoconstriction and carotid body responses to hypoxia.

Neural stem cell development

NDUFS2 is essential for glia-like neural stem and progenitor proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation.

Background

NDUFS2 frequently asked questions

What does NDUFS2 do?

NDUFS2 is a core subunit of mitochondrial Complex I, which transfers electrons from NADH to ubiquinone. It is essential for Complex I catalytic activity and assembly, and it also contributes to pulmonary and carotid body oxygen sensing. Check the datasheet of any product for its validated reactivity.

What other names does NDUFS2 go by, and does it have isoforms?

NDUFS2 is also known as CI-49, and its protein is named NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial. Earlier names refer to it as the Fe-S protein 2 (49kD). NCBI Gene reports alternatively spliced transcript variants encoding different isoforms.

Is NDUFS2 linked to disease and conserved in rodents?

Mutations in NDUFS2 are associated with mitochondrial complex I deficiency. The gene has orthologues in mouse and rat, both named Ndufs2, so rodent models can be used to study its role in Complex I function.

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Related targets

Other Complex I iron-sulfur and core subunits that act alongside NDUFS2 in the mitochondrial respiratory chain.

Gene and protein annotations from HGNC, NCBI Gene and UniProt O75306; product specifications from Assay Genie datasheets.

NDUFS2 Product listing