NDUFS3

Chr 11

NADH:ubiquinone oxidoreductase core subunit S3

Also known as: CI-30, MC1DN8

This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.[provided by RefSeq, Apr 2009]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial complex I deficiency, nuclear type 8

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARModerateconsider for supplementary testing2 gene-disease associations curated in total
0
Active trials
31
Pubs (1 yr)
P/LP submissions
P/LP missense
1.03
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.03LOEUF
pLI 0.001
Z-score 1.50
OE 0.55 (0.311.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.02Z-score
OE missense 1.00 (0.881.15)
150 obs / 149.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.55 (0.311.03)
00.351.4
Missense OE?1.00 (0.881.15)
00.61.4
Synonymous OE?1.13
01.21.6
LoF obs/exp: 7 / 12.8Missense obs/exp: 150 / 149.5Syn Z: -0.77

ClinVar

No ClinVar data available.

Protein Context — Lollipop Plot

NDUFS3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →