MT-ND1

Chr mitochondria

mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 1

Aliases:: ND1, NAD1

OMIM: 516000 ↗NCBI: 4535 ↗Ensembl ↗

Annotations refreshed 9 hours ago.

Predicted protein structure

AlphaFold predicted structure

MT-ND1 · P03886

v62025-08-01

Predicted Aligned Error matrix for MT-ND1 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

91.8/ 100

Very high

318 residues

Confidence breakdown

Very high(≥ 90)80%
Confident(70–90)19%
Low(50–70)2%
Very low(< 50)0%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Likely inborn error of metabolism
MITOCHONDRIAL
Mitochondrial disorders
MITOCHONDRIAL
Optic neuropathy
MITOCHONDRIAL
Structural basal ganglia disorders
MITOCHONDRIAL
Undiagnosed metabolic disorders
MITOCHONDRIAL
Adult onset dystonia, chorea or related movement disorder
MITOCHONDRIAL
Intellectual disability
MITOCHONDRIAL
Retinal disorders
MITOCHONDRIAL

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Disease associations (Open Targets)

Leber hereditary optic neuropathy
0.75
MELAS syndrome
0.73
mitochondrial disease
0.70
type 2 diabetes mellitus
0.66
mitochondrial complex I deficiency
0.66
MELAS
0.64
sudden infant death syndrome
0.62
mitochondrial non-syndromic sensorineural hearing loss
0.59
diabetes mellitus
0.58
Leigh syndrome
0.58

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

NADH-ubiquinone oxidoreductase chain 1

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:1959619). Essential for the catalytic activity and assembly of complex I (PubMed:1959619, PubMed:26929434)

Curated MONDO disease pages that list MT-ND1 among their top associated genes.

Predicted protein structure

Clinical relevance (Genomics England PanelApp)

Disease associations (Open Targets)

Protein function (UniProt)

Diseases associated with MT-ND1