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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GET3
guided entry of tail-anchored proteins factor 3, ATPase
Chromosome 19 · 19p13.13
NCBI Gene: 439Ensembl: ENSG00000198356.13HGNC: HGNC:752UniProt: O43681
100PubMed Papers
21Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingprotein carrier activitytail-anchored membrane protein insertion into ER membranenucleoplasmcardiomyopathy, dilated, 2Hfamilial isolated dilated cardiomyopathyFamilial prostate cancerprostate cancer
✦AI Summary

GET3 is an ATPase that functions as the central component of the GET (guided entry of tail-anchored proteins) pathway, mediating post-translational delivery of tail-anchored (TA) proteins to the endoplasmic reticulum 1. GET3 recognizes and selectively binds the transmembrane domain of nascent TA proteins in the cytosol through a hydrophobic groove 2. The protein operates through a nucleotide-dependent cycle: ATP binding drives GET3 toward a closed conformation facilitating TA protein recognition, while ATP hydrolysis enables transfer of the substrate to the membrane-bound GET1/CAMLG receptor complex 3. Following insertion, GET3 reverts to its open conformation, lowering receptor affinity and allowing cytosolic recycling 4. The GET1/Get2 insertase forms an aqueous channel approximately 2.5 nm in diameter that mediates TMD insertion into the lipid bilayer, with GET3 binding capable of sealing this channel 5. GET3 also exhibits chaperone holdase activity independent of its targeting function 2. Clinically, mutations in GET3 cause dilated cardiomyopathy 2H 6, while defects in the GET pathway components, including GET4, cause congenital disorders of glycosylation with neurological manifestations, highlighting GET3's importance in protein biogenesis and organellar function.

Sources cited
1
GET3 is an ATPase required for post-translational delivery of TA proteins to the ER
PMID: 17382883
2
GET3 has a hydrophobic groove for TA protein binding and possesses chaperone holdase activity
PMID: 30972921
3
ATP binding and hydrolysis drive GET3 conformational changes for client recognition and insertion
PMID: 35851188
4
GET1/Get2 insertase complex undergoes conformational changes driven by GET3 interaction to promote substrate insertion
PMID: 37963916
5
GET1/Get2 forms an aqueous channel for TA protein insertion, with GET3 binding sealing the channel
PMID: 36640319
6
GET3 deficiency causes dilated cardiomyopathy 2H and affects glycosylation and SNARE assembly
PMID: 35262690
Disease Associationsⓘ21
cardiomyopathy, dilated, 2HOpen Targets
0.50Moderate
familial isolated dilated cardiomyopathyOpen Targets
0.37Weak
Familial prostate cancerOpen Targets
0.11Weak
prostate cancerOpen Targets
0.11Weak
type 2 diabetes mellitusOpen Targets
0.02Suggestive
viral diseaseOpen Targets
0.02Suggestive
cardiomyopathyOpen Targets
0.02Suggestive
infectionOpen Targets
0.01Suggestive
heart diseaseOpen Targets
0.01Suggestive
melanomaOpen Targets
0.01Suggestive
ovarian cancerOpen Targets
0.01Suggestive
Emery-Dreifuss muscular dystrophyOpen Targets
0.01Suggestive
diabetes mellitusOpen Targets
0.01Suggestive
breast adenomaOpen Targets
0.01Suggestive
carcinomaOpen Targets
0.01Suggestive
dilated cardiomyopathyOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
pancreatic agenesisOpen Targets
0.00Suggestive
Cardiomyopathy, dilated, 2HUniProt
Pathogenic Variants1
NM_004317.4(GET3):c.488T>C (p.Val163Ala)Pathogenic
Cardiomyopathy, dilated, 2H
☆☆☆☆2025→ Residue 163
View on ClinVar ↗
Related Genes
UBL4AProtein interaction100%VAMP2Protein interaction100%SEC61A1Protein interaction100%BAG6Protein interaction99%SGTAProtein interaction98%RNF126Protein interaction96%
Tissue Expression6 tissues
Heart
100%
Brain
85%
Bone Marrow
85%
Lung
69%
Ovary
59%
Liver
58%
Gene Interaction Network
Click a node to explore
GET3UBL4AVAMP2SEC61A1BAG6SGTARNF126
PROTEIN STRUCTURE
Preparing viewer…
PDB8CQZ · 2.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.73LoF Tolerant
pLIⓘ
0.03Tolerant
Observed/Expected LoF0.48 [0.32–0.73]
RankingsWhere GET3 stands among ~20K protein-coding genes
  • #4,781of 20,598
    Most Researched100 · top quartile
  • #4,886of 5,498
    Most Pathogenic Variants1
  • #5,721of 17,882
    Most Constrained (LOEUF)0.73
Genes detectedGET3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The natural history of Get3-like chaperones.
PMID: 30972921
Traffic · 2019
1.00
2
The GET insertase exhibits conformational plasticity and induces membrane thinning.
PMID: 37963916
Nat Commun · 2023
0.90
3
Structural insights into metazoan pretargeting GET complexes.
PMID: 34887561
Nat Struct Mol Biol · 2021
0.80
4
Structurally derived universal mechanism for the catalytic cycle of the tail-anchored targeting factor Get3.
PMID: 35851188
Nat Struct Mol Biol · 2022
0.70
5
The Get1/2 insertase forms a channel to mediate the insertion of tail-anchored proteins into the ER.
PMID: 36640319
Cell Rep · 2023
0.60