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.
Other Names
mitochondrially encoded NADH dehydrogenase 4; MTND4; NADH dehydrogenase 4; NADH dehydrogenase subunit 4; NADH-ubiquinone oxidoreductase chain 4; NADH-ubiquinone oxidoreductase, subunit ND4; ND4; NU4M_HUMAN
Related Genetics Topics
- Leber hereditary optic neuropathy — MedlinePlus Genetics
- Leigh syndrome — MedlinePlus Genetics
- Mitochondrial complex I deficiency — MedlinePlus Genetics
Identifiers
- NCBI Gene: 4538
- OMIM: 256000
- OMIM: 516003
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
