NDUFAF1

Chr 15

NADH:ubiquinone oxidoreductase complex assembly factor 1

Also known as: CGI-65, CGI65, CIA30, MC1DN11

This gene encodes a complex I assembly factor protein. Complex I (NADH-ubiquinone oxidoreductase) catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q) in the first step of the mitochondrial respiratory chain, resulting in the translocation of protons across the inner mitochondrial membrane. The encoded protein is required for assembly of complex I, and mutations in this gene are a cause of mitochondrial complex I deficiency. Alternatively spliced transcript variants have been observed for this gene, and a pseudogene of this gene is located on the long arm of chromosome 19. [provided by RefSeq, Dec 2011]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial complex I deficiency, nuclear type 11

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARModerateconsider for supplementary testing
0
Active trials
8
Pubs (1 yr)
P/LP submissions
P/LP missense
0.91
LOEUF
DN
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?
0.91LOEUF
pLI 0.004
Z-score 1.82
OE 0.46 (0.250.91)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.44Z-score
OE missense 1.09 (0.971.23)
198 obs / 181.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.46 (0.250.91)
00.351.4
Missense OE?1.09 (0.971.23)
00.61.4
Synonymous OE?0.84
01.21.6
LoF obs/exp: 6 / 13.1Missense obs/exp: 198 / 181.3Syn Z: 1.04

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NDUFAF1 · 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.

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