C16ORF96

Chr 16

chromosome 16 open reading frame 96

This gene encodes a protein of unknown function that localizes to the mitochondria. Biallelic mutations cause a severe early-onset mitochondrial disorder characterized by developmental delay, seizures, hypotonia, and metabolic dysfunction. The disorder follows autosomal recessive inheritance and the gene shows minimal constraint against loss-of-function variants.

LOEUF 1.08
Clinical SummaryC16ORF96
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
1.08LOEUF
pLI 0.000
Z-score 1.14
OE 0.81 (0.621.08)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.77Z-score
OE missense 0.92 (0.850.98)
599 obs / 654.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.81 (0.621.08)
00.351.4
Missense OE0.92 (0.850.98)
00.61.4
Synonymous OE0.76
01.21.6
LoF obs/exp: 36 / 44.2Missense obs/exp: 599 / 654.3Syn Z: 3.17

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

C16ORF96 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found