NDUFAF6

Chr 8

NADH:ubiquinone oxidoreductase complex assembly factor 6

Also known as: C8orf38, FRTS5, MC1DN17, lncREST

This gene encodes a protein that localizes to mitochondria and contains a predicted phytoene synthase domain. The encoded protein plays an important role in the assembly of complex I (NADH-ubiquinone oxidoreductase) of the mitochondrial respiratory chain through regulation of subunit ND1 biogenesis. Mutations in this gene are associated with complex I enzymatic deficiency. [provided by RefSeq, Nov 2011]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial complex I deficiency, nuclear type 17
UniProtFanconi renotubular syndrome 5

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
0
Active trials
12
Pubs (1 yr)
P/LP submissions
P/LP missense
1.20
LOEUF
DN
Mechanism· predicted
📖
GeneReview available — NDUFAF6
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
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?
1.20LOEUF
pLI 0.000
Z-score 0.95
OE 0.75 (0.491.20)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.57Z-score
OE missense 0.87 (0.761.00)
142 obs / 162.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.75 (0.491.20)
00.351.4
Missense OE?0.87 (0.761.00)
00.61.4
Synonymous OE?0.82
01.21.6
LoF obs/exp: 13 / 17.3Missense obs/exp: 142 / 162.5Syn Z: 1.09

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NDUFAF6 · 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 →