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GeneE
9 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ZER1
zyg-11 related cell cycle regulator
Chromosome 9 · 9q34.11
NCBI Gene: 10444Ensembl: ENSG00000160445.12HGNC: HGNC:30960UniProt: Q7Z7L7
33PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingprotein quality control for misfolded or incompletely synthesized proteinspositive regulation of proteasomal ubiquitin-dependent protein catabolic processCul2-RING ubiquitin ligase complexalcohol drinkingcancercervical canceruterine cancer
✦AI Summary

ZER1 (zyg-11 related cell cycle regulator) is a substrate adapter subunit of the Cullin2-RING E3 ubiquitin ligase complex (CRL2ZER1) 1. It functions redundantly with ZYG11B to recognize N-terminal glycine (Gly/N-degron) degrons—the first four residues of substrate proteins—and target them for proteasomal degradation 2. ZER1 utilizes its armadillo repeat domain to form a deep binding cavity accommodating glycine through conserved hydrogen bonds and electrostatic interactions 3. ZER1 operates in multiple cellular contexts. During apoptosis, it mediates clearance of caspase-generated proteolytic fragments bearing N-terminal glycine degrons enriched at cleavage sites 1. It also participates in protein quality control by targeting misfolded N-myristoylated proteins when myristoylation fails, conditionally exposing glycine degrons 1. Pathologically, ZER1 contributes to HPV16-mediated carcinogenesis by interacting with the E7 oncoprotein and facilitating retinoblastoma protein (RB1) degradation—ZER1 is essential for growth in HPV-positive cancer cell lines but dispensable in HPV-negative lines 4. ZER1's specificity extends to therapeutic applications, as glycine-based PROTACs exploiting CRL2ZER1 effectively degrade oncogenic kinases like EML4-ALK and EGFR in lung cancer models 5. These findings establish ZER1 as a critical regulator of protein homeostasis with substantial clinical relevance in both viral oncogenesis and targeted protein degradation strategies.

Sources cited
1
ZER1 is a substrate adapter of Cul2ZER1 E3 ligase complex targeting N-terminal glycine; involved in caspase cleavage fragment clearance and N-myristoylation quality control
PMID: 31273098
2
Structural basis showing ZER1 uses armadillo repeats to recognize first four residues of Gly/N-degrons through conserved hydrogen bonds
PMID: 34214466
3
Molecular dynamics study revealing van der Waals and electrostatic interactions drive ZER1-peptide binding with specific key residues enabling recognition
PMID: 40108959
4
ZER1 interacts with HPV16 E7 oncoprotein, contributes to RB1 degradation, and is essential for growth of HPV-positive cancer cells
PMID: 36346242
5
Glycine-based PROTACs recruiting CRL2ZER1 effectively degrade oncogenic kinases EML4-ALK and EGFR in NSCLC
PMID: 39114932
6
ZER1/ZYG11B complex mediates N-glycine-dependent degradation of NLRP1 autoinhibitory fragment after 3C protease cleavage
PMID: 33093214
7
ZER1 specifically interacts with HPV16 E7 and is required for RB1 destabilization in HPV16 E7-expressing cells
PMID: 22232672
Disease Associationsⓘ20
alcohol drinkingOpen Targets
0.11Weak
cancerOpen Targets
0.04Suggestive
cervical cancerOpen Targets
0.03Suggestive
uterine cancerOpen Targets
0.00Suggestive
chronic kidney diseaseOpen Targets
0.00Suggestive
non-small cell lung carcinomaOpen Targets
0.00Suggestive
Ehlers-Danlos syndromeOpen Targets
0.00Suggestive
triple-negative breast cancerOpen Targets
0.00Suggestive
spina bifidaOpen Targets
0.00Suggestive
esophageal cancerOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
hereditary breast carcinomaOpen Targets
0.00Suggestive
lung cancerOpen Targets
0.00Suggestive
lymphomaOpen Targets
0.00Suggestive
melanomaOpen Targets
0.00Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.00Suggestive
sarcomaOpen Targets
0.00Suggestive
ThymomaOpen Targets
0.00Suggestive
thyroid cancer, nonmedullary, 1Open Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KLHDC3Protein interaction95%KLHDC2Protein interaction95%ELOCProtein interaction95%FEM1CProtein interaction94%PRAMEF6Protein interaction94%KLHDC10Protein interaction93%
Tissue Expression6 tissues
Heart
100%
Brain
96%
Ovary
66%
Liver
51%
Lung
48%
Bone Marrow
37%
Gene Interaction Network
Click a node to explore
ZER1KLHDC3KLHDC2ELOCFEM1CPRAMEF6KLHDC10
PROTEIN STRUCTURE
Preparing viewer…
PDB7XYT · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.24Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.15 [0.09–0.24]
RankingsWhere ZER1 stands among ~20K protein-coding genes
  • #11,449of 20,598
    Most Researched33
  • #711of 17,882
    Most Constrained (LOEUF)0.24 · top 5%
Genes detectedZER1
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
A glycine-specific N-degron pathway mediates the quality control of protein
PMID: 31273098
Science · 2019
1.00
2
Distinct Amino Acid-Based PROTACs Target Oncogenic Kinases for Degradation in Non-Small Cell Lung Cancer (NSCLC).
PMID: 39114932
J Med Chem · 2024
0.89
3
Molecular basis for recognition of Gly/N-degrons by CRL2
PMID: 34214466
Mol Cell · 2021
0.78
4
Enteroviral 3C protease activates the human NLRP1 inflammasome in airway epithelia.
PMID: 33093214
Science · 2020
0.67
5
Molecular mechanism of ZER1-mediated recognition of N-terminal glycine in protein degradation.
PMID: 40108959
J Biomol Struct Dyn · 2026
0.56