NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10

NDUFB10 (NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10) is an accessory subunit of mitochondrial respiratory chain complex I that facilitates functional assembly of the complex and mediates electron transfer from NADH to ubiquinone (UniProt: O96000). The protein is essential for oxidative phosphorylation and cellular energy metabolism.

NDUFB10 is expressed in mitochondria across tissues requiring high ATP turnover. Pathogenic variants in NDUFB10 cause mitochondrial complex I deficiency, nuclear type 35 (MC1DN35), an autosomal recessive disorder characterized by defective oxidative phosphorylation with variable clinical severity ranging from neonatal lethality to adult-onset neurodegeneration, including Leigh syndrome, leukodystrophy, and Parkinson disease (UniProt: O96000).

NDUFB10 is associated with Alzheimer's Disease and shows significant upregulation in human post-mortem AD brain compared to age-matched controls (mean log2FC = 1.74, Chaparral AD proteomics). This upregulation may reflect compensatory mitochondrial responses or altered energy metabolism in AD pathology, though the functional significance requires further investigation.