### TMEM126B Gene Overview

**Gene Symbol:** TMEM126B  
**Location:** Human Gene Map — Chromosome 2

#### 1. Associated Genes
TMEM126B is known to have associations with several genes involved in mitochondrial processes and energy metabolism, specifically:

- **NDUFAF13 (NADH dehydrogenase (ubiquinone) 1 alpha subcomplex assembly factor 13):** Directly interacts as noted; plays a role in the assembly of complex I.
- **NDUFS1, NDUFS2, and other NDUF-related genes:** These genes are components of the mitochondrial complex I (NADH:ubiquinone oxidoreductase), and improper assembly can lead to deficiencies and mitochondrial disorders.
- **Other mitochondrial assembly factors:** Various genes implicated in the assembly and function of mitochondrial complexes may relate to TMEM126B.

#### 2. Aging and Age-Related Disease Association
While explicit details connecting TMEM126B directly to aging are not deeply characterized, mitochondrial dysfunction and impaired complex I assembly, which TMEM126B is involved in, have been linked to several age-related disorders. Additionally:

- **Mitochondrial Dysfunction and Aging:** As aging progresses, mitochondrial function tends to decline, leading to decreased energy production and increased oxidative stress. This is a common feature seen in age-related degenerative diseases.
- **Neurological Disorders:** Mutations in TMEM126B are associated with isolated complex I deficiencies, which may correlate with pathogenesis in neurodegenerative diseases, such as Parkinson's disease or Alzheimer's disease. Impaired mitochondrial function has been implicated in the aging process and these neurodegenerative conditions.

#### 3. Drug Interactions
Currently, there are limited specific drugs or drug classes directly associated with TMEM126B. However, general mitochondrial function modulators can possibly affect this gene's pathway, which include:

- **Mitochondrial antioxidants:** Such as Coenzyme Q10 (ubiquinone) may aid in protecting complex I activity.
- **Investigational drugs:** Potential therapies targeting mitochondrial respiration or bioenergetics might interact indirectly with TMEM126B-related pathways.
  
It is important to note that drug interactions with TMEM126B are not well characterized, thus further research is necessary to clarify potential pharmacogenomic interactions.

#### 4. Pathways and Biological Processes
TMEM126B is involved in various key biological pathways, particularly related to mitochondrial function:

- **Mitochondrial Complex I Assembly:** As a component of the mitochondrial complex I assembly complex (MCIA), TMEM126B is crucial in the photoproduction, membrane arm formation, and assembly of NADH:ubiquinone oxidoreductase.
- **Mitochondrial Respiration:** Through its role in complex I, TMEM126B significantly contributes to the electron transport chain, cellular respiration, and ATP production.
- **Cellular Metabolism:** By enabling proper assembly of complex I, TMEM126B indirectly influences metabolic pathways related to energy production and reactive oxygen species handling.

In conclusion, TMEM126B is an essential gene for mitochondrial function, particularly in the assembly of complex I, with implications in age-related diseases and potential therapeutic relevance in mitochondrial dysfunction disorders. Further research may illuminate more specific associations with pharmacological agents as well as deeper insights regarding aging processes.