| NDUFA5 — NADH:ubiquinone Oxidoreductase Subunit A5 | |
|---|---|
| Symbol | NDUFA5 |
| Full Name | NADH:ubiquinone Oxidoreductase Subunit A5 (B13, Complex I 13kDa) |
| Chromosome | 7q31.32 |
| NCBI Gene | 4698 |
| Ensembl | ENSG00000167863 |
| UniProt | P06540 |
| Diseases | [PD](/diseases/parkinsons-disease), [AD](/diseases/alzheimers), [ALS](/diseases/als), Leigh syndrome, mitochondrial complex I deficiency |
| Expression | Ubiquitous (high in brain, heart, muscle) |
NDUFA5 encodes the B13 subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase), a critical component of the electron transport chain[1][2]. This page covers its function and role in neurodegenerative diseases.
NDUFA5 (also known as B13, NUFM, UQOR13, CI-13kB) is located on chromosome 7q31.32 and encodes a 116-amino acid protein that is part of the Q module of mitochondrial Complex I[3]. Complex I is the largest enzyme of the mitochondrial respiratory chain, containing 45 subunits.
NDUFA5 is essential for:
The protein supports:
Complex I is a major source of reactive oxygen species (ROS). NDUFA5 function affects cellular oxidative stress.
NDUFA5 is expressed ubiquitously with highest levels in:
[Fearnley & Walker. Conservation of components of the mitochondrial complex I](https://doi.org/10.1016/0968-0004(92). Trends in Biochemical Sciences. 1992. ↩︎
Sazanov. A 3.3 Å resolution structure of human mitochondrial respiratory chain complex I: implications for the mechanism. Biochimica et Biophysica Acta. 2015. ↩︎
Koopman et al. Mammalian mitochondrial complex I: biogenesis, genetics, and disease. Journal of Medical Genetics. 2013. ↩︎