ATG4C

Chr 1

autophagy related 4C cysteine peptidase

Also known as: APG4-C, APG4C, AUTL1, AUTL3, HsAPG4C

ATG4C encodes a cysteine protease that mediates both proteolytic activation and delipidation of ATG8 family proteins, which are essential steps in the autophagy pathway for cellular homeostasis and organelle turnover. The gene shows very low constraint against loss-of-function variants (pLI near zero, LOEUF 1.4), and no definitive human disease associations have been established with ATG4C mutations to date.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.41
Clinical SummaryATG4C
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
21 unique Pathogenic / Likely Pathogenic· 76 VUS of 115 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.41LOEUF
pLI 0.000
Z-score -0.03
OE 1.01 (0.731.41)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.44Z-score
OE missense 1.08 (0.971.20)
244 obs / 225.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.01 (0.731.41)
00.351.4
Missense OE1.08 (0.971.20)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 25 / 24.9Missense obs/exp: 244 / 225.5Syn Z: -0.06

ClinVar Variant Classifications

115 submitted variants in ClinVar

Classification Summary

Pathogenic20
Likely Pathogenic1
VUS76
Likely Benign1
20
Pathogenic
1
Likely Pathogenic
76
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
20
0
20
Likely Pathogenic
0
0
1
0
1
VUS
0
68
8
0
76
Likely Benign
0
1
0
0
1
Benign
0
0
0
0
0
Total06929098

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ATG4C · 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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