NDUFAF5

Chr 20AR

NADH:ubiquinone oxidoreductase complex assembly factor 5

Also known as: C20orf7, MC1DN16, bA526K24.2, dJ842G6.1

The protein is required for assembly of mitochondrial respiratory chain complex I, which catalyzes electron transfer from NADH to ubiquinone. Mutations cause mitochondrial complex I deficiency, nuclear type 16, inherited in an autosomal recessive pattern. The pathogenic mechanism involves impaired complex I assembly leading to deficient mitochondrial respiratory chain function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 1.201 OMIM phenotype
Clinical SummaryNDUFAF5
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Gene-Disease Validity (ClinGen)
Leigh syndrome · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

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 0.85
OE 0.80 (0.551.20)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.15Z-score
OE missense 0.97 (0.861.09)
187 obs / 192.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.80 (0.551.20)
00.351.4
Missense OE0.97 (0.861.09)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 17 / 21.2Missense obs/exp: 187 / 192.9Syn Z: -0.41

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NDUFAF5 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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