NDUFV2

Chr 18

NADH:ubiquinone oxidoreductase core subunit V2

Also known as: CI-24k, MC1DN7

The NADH-ubiquinone oxidoreductase complex (complex I) of the mitochondrial respiratory chain catalyzes the transfer of electrons from NADH to ubiquinone, and consists of at least 43 subunits. The complex is located in the inner mitochondrial membrane. This gene encodes the 24 kDa subunit of complex I, and is involved in electron transfer. Mutations in this gene are implicated in Parkinson's disease, bipolar disorder, schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. A non-transcribed pseudogene of this locus is found on chromosome 19. [provided by RefSeq, Oct 2009]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial complex I deficiency, nuclear type 7

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
0
Active trials
23
Pubs (1 yr)
P/LP submissions
P/LP missense
0.83
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?
0.83LOEUF
pLI 0.006
Z-score 2.02
OE 0.42 (0.230.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.55Z-score
OE missense 1.14 (0.991.30)
149 obs / 131.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.42 (0.230.83)
00.351.4
Missense OE?1.14 (0.991.30)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 6 / 14.2Missense obs/exp: 149 / 131.1Syn Z: 0.07

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NDUFV2 · 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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