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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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TEX264
testis expressed 264, ER-phagy receptor
Chromosome 3 · 3p21.2
NCBI Gene: 51368Ensembl: ENSG00000164081.13HGNC: HGNC:30247UniProt: A0A087WTU3
66PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA Repair
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
reticulophagyprotein bindingsignaling receptor activityprotein-DNA covalent cross-linking repairchondrocalcinosisasthmaAbnormality of the skeletal systemmetabolic syndrome
✦AI Summary

TEX264 is a major endoplasmic reticulum (ER)-resident reticulophagy receptor that mediates selective autophagic degradation of ER subdomains 1. As an ER protein with a single transmembrane domain and an LC3-interacting region (LIR) motif, TEX264 interacts with LC3 and GABARAP family proteins to bridge ER and autophagosomal membranes 2. During nutrient stress, TEX264 recognizes tubular ER segments near three-way junctions and recruits them into ATG8-containing isolation membranes through trans interactions, facilitating autophagosomal enclosure and lysosomal fusion for ER turnover 1. An intrinsically disordered region enables its ER-phagy function independent of amino acid sequence 2. Beyond ER homeostasis, TEX264 functions in DNA repair by sensing topoisomerase 1 cleavage complexes (TOP1cc) at replication forks and coordinating their resolution with the p97 ATPase and SPRTN metalloprotease 3. This dual role promotes lysosomal processing of TOP1cc lesions and facilitates cell survival after topoisomerase inhibition 4. TEX264-mediated reticulophagy also contributes to autophagic cell death responses under ER stress conditions 5, with potential implications for viral defense mechanisms 6. These multifaceted functions establish TEX264 as a critical regulator of ER quality control and genome stability.

Sources cited
1
TEX264 is an ER-resident reticulophagy receptor with LIR motif that acts independently to remodel ER during nutrient stress through ATG8 interactions at three-way junctions
PMID: 31006537
2
TEX264 is identified as a major ER-phagy receptor with intrinsically disordered region required for bridging ER and autophagosomal membranes, more efficient than previously known ER-phagy receptors
PMID: 31006538
3
TEX264 coordinates p97 and SPRTN-mediated resolution of topoisomerase 1-DNA adducts by recognizing TOP1cc and recruiting proteolytic machinery at DNA replication forks
PMID: 32152270
4
TEX264 acts as a TOP1cc sensor at replication forks, triggering autophagy-mediated lysosomal processing of DNA lesions to promote DNA repair and cell survival
PMID: 39265577
5
TEX264 mediates reticulophagy in response to ER stress and contributes to autophagic cell death, downstream of ATF4 transcriptional activation
PMID: 33111629
6
TEX264 reticulophagy machinery serves as potential antiviral defense mechanism that can be subverted by viruses
PMID: 39394962
Disease Associationsⓘ20
chondrocalcinosisOpen Targets
0.27Weak
asthmaOpen Targets
0.14Weak
Abnormality of the skeletal systemOpen Targets
0.12Weak
metabolic syndromeOpen Targets
0.07Suggestive
Abnormality of refractionOpen Targets
0.02Suggestive
spermatoceleOpen Targets
0.02Suggestive
mental or behavioural disorderOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
Darier diseaseOpen Targets
0.01Suggestive
lung cancerOpen Targets
0.01Suggestive
insomniaOpen Targets
0.01Suggestive
acute kidney injuryOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
chronic kidney diseaseOpen Targets
0.00Suggestive
posterior cortical atrophyOpen Targets
0.00Suggestive
triple-negative breast cancerOpen Targets
0.00Suggestive
Chédiak-Higashi syndromeOpen Targets
0.00Suggestive
in situ carcinomaOpen Targets
0.00Suggestive
infectionOpen Targets
0.00Suggestive
Salmonella InfectionsOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GABARAPProtein interaction96%RTN3Protein interaction72%RETREG1Protein interaction61%ATL3Protein interaction60%CCPG1Protein interaction53%SEC62Protein interaction50%
Tissue Expression6 tissues
Liver
100%
Heart
66%
Ovary
53%
Lung
50%
Bone Marrow
41%
Brain
29%
Gene Interaction Network
Click a node to explore
TEX264GABARAPRTN3RETREG1ATL3CCPG1SEC62
PROTEIN STRUCTURE
Preparing viewer…
PDB7VED · 2.02 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.80LoF Tolerant
pLIⓘ
0.02Tolerant
Observed/Expected LoF0.50 [0.33–0.80]
RankingsWhere TEX264 stands among ~20K protein-coding genes
  • #7,127of 20,598
    Most Researched66
  • #6,631of 17,882
    Most Constrained (LOEUF)0.80
Genes detectedTEX264
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Intrinsically Disordered Protein TEX264 Mediates ER-phagy.
PMID: 31006538
Mol Cell · 2019
1.00
2
Reticulophagy and viral infection.
PMID: 39394962
Autophagy · 2025
0.90
3
ATF4 links ER stress with reticulophagy in glioblastoma cells.
PMID: 33111629
Autophagy · 2021
0.80
4
USP20 deubiquitinates and stabilizes the reticulophagy receptor RETREG1/FAM134B to drive reticulophagy.
PMID: 38705724
Autophagy · 2024
0.70
5
TEX264 drives selective autophagy of DNA lesions to promote DNA repair and cell survival.
PMID: 39265577
Cell · 2024
0.60