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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ATL2
atlastin GTPase 2
Chromosome 2 · 2p22.2-p22.1
NCBI Gene: 64225Ensembl: ENSG00000119787.14HGNC: HGNC:24047UniProt: B5MCN0
50PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
endoplasmic reticulum tubular network membraneendoplasmic reticulumendoplasmic reticulum membranemembraneneurodegenerative diseasedengue diseaseliver diseasecervical carcinoma
✦AI Summary

ATL2 (atlastin GTPase 2) is a membrane-anchored dynamin-like GTPase that mediates GTP-dependent fusion of endoplasmic reticulum (ER) membranes, maintaining the continuous ER network and forming three-way junctions where ER tubules intersect 1. The fusion mechanism involves homodimer formation through G domains upon GTP binding, followed by conformational tightening of the three-helix bundle regions that pulls membranes together, with subsequent GTP hydrolysis and dimer disassembly completing the cycle 2. ATL2 exists as multiple splice isoforms with divergent regulatory properties; the major isoform ATL2-1 is autoinhibited by its C-terminal α-helix, while neuronal isoforms show full fusion activity 1. All human atlastins, including ATL2, are sufficient to drive liposome fusion with physiological ER lipid composition 3. Beyond ER morphology, ATL2/3 regulate ULK1 complex targeting to the ER to initiate autophagy 4. Clinically, high ATL2-2 expression associates with worse prognosis in estrogen-receptor-positive breast cancer, correlating with MYC and E2F target pathway activation 5. Mutations in ATL genes are linked to hereditary sensory neuropathies and hereditary spastic paraplegia 2, highlighting ATL2's critical importance in cellular and neuronal health.

Sources cited
1
ATL2 mediates ER fusion through homodimer formation and GTP hydrolysis; major splice isoforms show autoinhibition by C-terminal α-helix
PMID: 34817557
2
ATLs are integral dynamin-like GTPases critical for ER network formation; humans express three paralogs with distinct expression patterns; mutations linked to hereditary sensory neuropathies and hereditary spastic paraplegia
PMID: 40587263
3
All human atlastins including ATL2 are sufficient to induce liposome fusion with physiological ER lipid composition
PMID: 36757370
4
ATL2/3 regulate ER targeting of the ULK1 complex to initiate autophagy and autophagosome formation
PMID: 33988678
5
High ATL2-2 expression associates with worse prognosis in estrogen-receptor-positive breast cancer and correlates with upregulation of MYC targets, E2F targets, and G2M checkpoint pathways
PMID: 37628611
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.53Moderate
dengue diseaseOpen Targets
0.37Weak
liver diseaseOpen Targets
0.32Weak
cervical carcinomaOpen Targets
0.32Weak
Alzheimer diseaseOpen Targets
0.28Weak
skin neoplasmOpen Targets
0.27Weak
bile duct disorderOpen Targets
0.27Weak
lysosomal storage diseaseOpen Targets
0.26Weak
multiple sclerosisOpen Targets
0.26Weak
Parkinson diseaseOpen Targets
0.26Weak
actinic keratosisOpen Targets
0.21Weak
elevated lactate dehydrogenaseOpen Targets
0.18Weak
placental retentionOpen Targets
0.08Suggestive
rheumatoid arthritisOpen Targets
0.07Suggestive
gastritisOpen Targets
0.07Suggestive
skin cancerOpen Targets
0.07Suggestive
Blackfan-Diamond anemiaOpen Targets
0.06Suggestive
cataractOpen Targets
0.06Suggestive
infectious meningitisOpen Targets
0.06Suggestive
trauma complicationOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATL3Shared pathway100%REEP1Protein interaction98%RTN1Protein interaction86%SPARTProtein interaction86%SSNA1Protein interaction83%CHMP1BProtein interaction83%
Tissue Expression6 tissues
Liver
100%
Ovary
94%
Bone Marrow
92%
Heart
57%
Lung
51%
Brain
37%
Gene Interaction Network
Click a node to explore
ATL2ATL3REEP1RTN1SPARTSSNA1CHMP1B
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8NHH9
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.65LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.47 [0.35–0.65]
RankingsWhere ATL2 stands among ~20K protein-coding genes
  • #8,774of 20,598
    Most Researched50
  • #4,672of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedATL2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Construction and Preclinical Evaluation of a
PMID: 36579764
Mol Pharm · 2023
1.00
2
High Atlastin 2-2 (ATL2-2) Expression Associates with Worse Prognosis in Estrogen-Receptor-Positive Breast Cancer.
PMID: 37628611
Genes (Basel) · 2023
0.90
3
Human atlastins are sufficient to drive the fusion of liposomes with a physiological lipid composition.
PMID: 36757370
J Cell Biol · 2023
0.80
4
Reconstitution of human atlastin fusion activity reveals autoinhibition by the C terminus.
PMID: 34817557
J Cell Biol · 2022
0.70
5
Connecting tubules: mechanisms of endoplasmic reticulum membrane fusion.
PMID: 40587263
Biochem Soc Trans · 2025
0.60