COX4I2

Chr 20

cytochrome c oxidase subunit 4I2

Also known as: COX4, COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2

Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may be involved in the regulation and assembly of the complex. This nuclear gene encodes isoform 2 of subunit IV. Isoform 1 of subunit IV is encoded by a different gene, however, the two genes show a similar structural organization. Subunit IV is the largest nuclear encoded subunit which plays a pivotal role in COX regulation. [provided by RefSeq, Jul 2008]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtExocrine pancreatic insufficiency dyserythropoietic anemia and calvarial hyperostosis

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARLimitednot for standalone diagnostic reporting
0
Active trials
15
Pubs (1 yr)
P/LP submissions
P/LP missense
1.34
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?
1.34LOEUF
pLI 0.000
Z-score 0.82
OE 0.72 (0.411.34)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.07Z-score
OE missense 1.02 (0.871.20)
105 obs / 103.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.72 (0.411.34)
00.351.4
Missense OE?1.02 (0.871.20)
00.61.4
Synonymous OE?1.08
01.21.6
LoF obs/exp: 7 / 9.8Missense obs/exp: 105 / 103.1Syn Z: -0.40

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

COX4I2 · 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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