ATG7

Chr 3AR

autophagy related 7

Also known as: APG7-LIKE, APG7L, GSA7, SCAR31

ATG7 encodes an E1-like activating enzyme essential for autophagy, activating ATG12 and ATG8 family proteins for conjugation reactions required for autophagosome formation and cytoplasm-to-vacuole transport. Mutations cause spinocerebellar ataxia, autosomal recessive 31, which follows autosomal recessive inheritance. The gene is not highly constrained against loss-of-function variants and plays critical roles in axonal homeostasis and mitochondrial quality control.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.941 OMIM phenotype
Clinical SummaryATG7
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Gene-Disease Validity (ClinGen)
spinocerebellar ataxia, autosomal recessive 31 · ARStrong

Strong evidence — appropriate for clinical testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.94LOEUF
pLI 0.000
Z-score 1.86
OE 0.66 (0.470.94)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.37Z-score
OE missense 0.80 (0.730.88)
313 obs / 388.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.66 (0.470.94)
00.351.4
Missense OE0.80 (0.730.88)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 23 / 34.8Missense obs/exp: 313 / 388.9Syn Z: 0.11
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongATG7-related intellectual disability and ataxiaLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6549th %ile
GOF
0.4974th %ile
LOF
0.3164th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ATG7 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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