C12ORF71

Chr 12

chromosome 12 open reading frame 71

The C12ORF71 protein is involved in mitochondrial ribosome assembly and function. Mutations cause autosomal recessive spastic paraplegia with optic atrophy, cerebellar ataxia, and neuropathy, typically presenting in childhood to early adulthood. The gene shows low constraint against loss-of-function variants, consistent with its recessive inheritance pattern.

0
Active trials
0
Pubs (1 yr)
33
P/LP submissions
P/LP missense
1.72
LOEUF
Mechanism
Clinical SummaryC12ORF71
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
33 unique Pathogenic / Likely Pathogenic· 2 VUS of 38 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.72LOEUF
pLI 0.003
Z-score 0.33
OE 0.84 (0.411.72)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.36Z-score
OE missense 0.91 (0.791.06)
132 obs / 144.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.84 (0.411.72)
00.351.4
Missense OE0.91 (0.791.06)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 4 / 4.8Missense obs/exp: 132 / 144.3Syn Z: -0.11

ClinVar Variant Classifications

38 submitted variants in ClinVar

Classification Summary

Pathogenic32
Likely Pathogenic1
VUS2
32
Pathogenic
1
Likely Pathogenic
2
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
32
Likely Pathogenic
1
VUS
2
Likely Benign
0
Benign
0
Total35

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

C12ORF71 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found