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Reviewed August 2006
What is the official name of the MT-ND4 gene?
The official name of this gene is “mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 4.”
MT-ND4 is the gene's official symbol. The MT-ND4 gene is also known by other names, listed below.
Read more about gene names and symbols on the About page.
What is the normal function of the MT-ND4 gene?
The MT-ND4 gene provides instructions for making a protein called NADH dehydrogenase 4. This protein is part of a large enzyme complex known as complex I, which is active in mitochondria. Mitochondria are structures within cells that convert the energy from food into a form that cells can use. These cellular structures produce energy through a process called oxidative phosphorylation, which uses oxygen and simple sugars to create adenosine triphosphate (ATP), the cell's main energy source.
Complex I is one of several enzyme complexes necessary for oxidative phosphorylation. Within mitochondria, these complexes are embedded in a tightly folded, specialized membrane called the inner mitochondrial membrane. During oxidative phosphorylation, mitochondrial enzyme complexes carry out chemical reactions that drive the production of ATP. Specifically, they create an unequal electrical charge on either side of the inner mitochondrial membrane through a step-by-step transfer of negatively charged particles called electrons. This difference in electrical charge provides the energy for ATP production.
Complex I is responsible for the first step in the electron transport process, the transfer of electrons from a molecule called NADH to another molecule called ubiquinone. Electrons are then passed from ubiquinone through several other enzyme complexes to provide energy for the generation of ATP.
How are changes in the MT-ND4 gene related to health conditions?
Genetics Home Reference provides information about Leigh syndrome, which is also associated with changes in the MT-ND4 gene.
Where is the MT-ND4 gene located?
The MT-ND4 gene is located in mitochondrial DNA.
Molecular Location in mitochondrial DNA: base pairs 10,760 to 12,137
(Homo sapiens Annotation Release 107, GRCh38.p2) (
Where can I find additional information about MT-ND4?
You and your healthcare professional may find the following resources about MT-ND4 helpful.
You may also be interested in these resources, which are designed for genetics professionals and researchers.
What other names do people use for the MT-ND4 gene or gene products?
See How are genetic conditions and genes named? in the Handbook.
Where can I find general information about genes?
The Handbook provides basic information about genetics in clear language.
These links provide additional genetics resources that may be useful.
What glossary definitions help with understanding MT-ND4?
adenosine triphosphate ; amino acid ; arginine ; ATP ; cell ; charged particles ; dehydrogenase ; electron ; enzyme ; gene ; hereditary ; histidine ; kidney ; mitochondria ; molecule ; mutation ; neuropathy ; optic nerve ; oxidative phosphorylation ; oxidoreductase ; oxygen ; phosphorylation ; protein ; reactive oxygen species ; serine ; subunit ; syndrome ; ubiquinone
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (10 links)
The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? in the Handbook.