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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ATG101
autophagy related 101
Chromosome 12 · 12q13.13
NCBI Gene: 60673Ensembl: ENSG00000123395.16HGNC: HGNC:25679UniProt: Q9BSB4
45PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingregulation of protein lipidationidentical protein bindingprotein-containing complex bindinginjurycancerneoplasmceliac disease
✦AI Summary

ATG101 (autophagy related 101) is an essential component of the autophagy initiation machinery in mammalian cells. ATG101 functions as a critical subunit of the ULK1 kinase complex, which comprises ULK1, ATG13, FIP200, and ATG101, and represents the most upstream regulatory complex in autophagy 1. The primary function of ATG101 is to stabilize ATG13 by protecting it from proteasomal degradation, thereby maintaining the integrity of the autophagy initiation complex 12. ATG101 interacts with ULK1 in an ATG13-dependent manner and is essential for macroautophagy 1. Mechanistically, ATG101 contains a newly identified WF motif that facilitates recruitment of downstream autophagy proteins 2. Loss of ATG101 function results in defective autophagy processes, including both developmental and starvation-induced autophagy, leading to tissue homeostasis disruption in adult brains and midguts 3. ATG101 deficiency causes accumulation of ubiquitin-positive aggregates, shortened lifespan, and mobility defects 3. Clinically, ATG101 has emerged as a prognostic biomarker in hepatocellular carcinoma, where ATG101-related signatures correlate with patient survival and therapeutic responses 4. The conservation of ATG101 across eukaryotes, except in yeast which has a 'capped' ATG13 that doesn't require ATG101 for stabilization, highlights its fundamental importance in autophagy regulation 2.

Sources cited
1
ATG101 is essential for macroautophagy, interacts with ULK1 and ATG13, and stabilizes ATG13 by protecting it from proteasomal degradation
PMID: 19287211
2
ATG101 stabilizes 'uncapped' ATG13 in most eukaryotes and contains a WF motif for recruiting downstream autophagy proteins
PMID: 26754330
3
ATG101 loss-of-function causes defective autophagy, tissue homeostasis disruption, ubiquitin aggregate accumulation, and shortened lifespan
PMID: 30760524
4
ATG101 serves as a prognostic biomarker in hepatocellular carcinoma with clinical significance for patient survival and therapeutic responses
PMID: 36302799
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
injuryOpen Targets
0.10Suggestive
cancerOpen Targets
0.09Suggestive
neoplasmOpen Targets
0.08Suggestive
celiac diseaseOpen Targets
0.04Suggestive
hepatocellular carcinomaOpen Targets
0.04Suggestive
infectious diseaseOpen Targets
0.04Suggestive
familial primary pulmonary hypoplasiaOpen Targets
0.03Suggestive
COVID-19Open Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
ischemiaOpen Targets
0.01Suggestive
systemic juvenile idiopathic arthritisOpen Targets
0.01Suggestive
adrenal cortex carcinomaOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
melanomaOpen Targets
0.01Suggestive
liver cancerOpen Targets
0.00Suggestive
cholangiocarcinomaOpen Targets
0.00Suggestive
diabetic nephropathyOpen Targets
0.00Suggestive
infectionOpen Targets
0.00Suggestive
malignant pleural mesotheliomaOpen Targets
0.00Suggestive
Nematoda infectious diseaseOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MTORProtein interaction100%NBR1Protein interaction100%PIK3C3Protein interaction100%UVRAGProtein interaction100%BECN1Protein interaction100%ATG12Protein interaction100%
Tissue Expression6 tissues
Brain
100%
Liver
96%
Lung
95%
Ovary
60%
Bone Marrow
55%
Heart
49%
Gene Interaction Network
Click a node to explore
ATG101MTORNBR1PIK3C3UVRAGBECN1ATG12
PROTEIN STRUCTURE
Preparing viewer…
PDB5C50 · 1.63 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.68LoF Tolerant
pLIⓘ
0.65Intermediate
Observed/Expected LoF0.35 [0.19–0.68]
RankingsWhere ATG101 stands among ~20K protein-coding genes
  • #9,413of 20,598
    Most Researched45
  • #5,075of 17,882
    Most Constrained (LOEUF)0.68
Genes detectedATG101
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Targeting autophagy-related protein kinases for potential therapeutic purpose.
PMID: 32322463
Acta Pharm Sin B · 2020
1.00
2
The autophagy-related gene
PMID: 30760524
J Biol Chem · 2019
0.90
3
Atg101: Not Just an Accessory Subunit in the Autophagy-initiation Complex.
PMID: 26754330
Cell Struct Funct · 2016
0.80
4
Palmitoylation of ULK1 by ZDHHC13 plays a crucial role in autophagy.
PMID: 39169022
Nat Commun · 2024
0.70
5
Metamorphosis by ATG13 and ATG101 in human autophagy initiation.
PMID: 37394799
Autophagy · 2024
0.60