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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HBE1
hemoglobin subunit epsilon 1
Chromosome 11 · 11p15.4
NCBI Gene: 3046Ensembl: ENSG00000213931.7HGNC: HGNC:4830UniProt: D9YZU7
108PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingblood microparticleoxygen transportoxygen carrier activitybeta thalassemiapneumoniacolorectal carcinomachronic hepatitis B virus infection
✦AI Summary

HBE1 encodes the epsilon globin chain, a beta-type hemoglobin subunit expressed during early mammalian embryonic development 1. This chain functions as part of embryonic hemoglobin, contributing to oxygen transport and binding 2. HBE1 expression is temporally regulated during fetal liver erythropoiesis, with increased expression in fetal liver-derived immortalized erythroid cells compared to cord blood cells, accompanied by downregulation of BCL11A and upregulation of LIN28B and IGF2BP1 2. Clinically, HBE1 variants are implicated in hemoglobinopathies. Deletions involving the HBE1 locus contribute to novel thalassemia categories; εγδβ-thalassemia (deletion of epsilon through beta-pseudogene loci) causes severe transient perinatal anemia, while εγ-thalassemia (deletion of epsilon through pseudobeta loci) presents with distinct phenotypes including increased HbA2 without microcytosis 3. Additionally, an HBE1 SNP (rs72872548) serves as a disease severity predictor in β0-thalassemia/HbE disease populations, demonstrating >85% specificity and 75% accuracy when combined with SNPs in BCL11A and HBS1L-MYB 4. HBE1 also shows signatures of positive selection in North African populations, suggesting adaptive significance in hemoglobin phenotypes 5.

Sources cited
1
HBE1 deletion causes εγδβ-thalassemia with severe transient perinatal anemia and εγ-thalassemia with increased HbA2 without microcytosis
PMID: 37086467
2
HBE1 SNP rs72872548 is a predictor of disease severity in β0-thalassemia/HbE disease
PMID: 33990643
3
HBE1 expression is upregulated in fetal liver-derived erythroid cells with altered BCL11A, LIN28B, and IGF2BP1 expression
PMID: 37657739
4
HBE1 is a beta-like globin gene; over 1,200 genetic alterations in globin genes including HBE1 cause hemoglobinopathy phenotypic heterogeneity
PMID: 16138310
5
HBE1 shows signatures of positive selection related to hemoglobin phenotypes in North African populations
PMID: 37210386
Disease Associationsⓘ20
beta thalassemiaOpen Targets
0.33Weak
pneumoniaOpen Targets
0.09Suggestive
colorectal carcinomaOpen Targets
0.05Suggestive
chronic hepatitis B virus infectionOpen Targets
0.03Suggestive
liver diseaseOpen Targets
0.03Suggestive
ThalassemiaOpen Targets
0.03Suggestive
hepatitis B virus infectionOpen Targets
0.03Suggestive
hepatocellular carcinomaOpen Targets
0.02Suggestive
colorectal cancerOpen Targets
0.02Suggestive
HepatitisOpen Targets
0.02Suggestive
chronic hepatitisOpen Targets
0.02Suggestive
infectionOpen Targets
0.02Suggestive
Chédiak-Higashi syndromeOpen Targets
0.02Suggestive
coinfectionOpen Targets
0.02Suggestive
cirrhosis of liverOpen Targets
0.01Suggestive
acute hepatitis B virus infectionOpen Targets
0.01Suggestive
Acute hepatitisOpen Targets
0.01Suggestive
leukemiaOpen Targets
0.01Suggestive
in situ carcinomaOpen Targets
0.01Suggestive
hemoglobinopathyOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HBDShared pathway100%HBA2Protein interaction97%AHSPProtein interaction94%HBG2Protein interaction94%HBZProtein interaction91%HBG1Protein interaction90%
Tissue Expression6 tissues
Ovary
100%
Brain
73%
Lung
54%
Liver
31%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
HBE1HBDHBA2AHSPHBG2HBZHBG1
PROTEIN STRUCTURE
Preparing viewer…
PDB1A9W · 2.90 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.59LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.93 [0.56–1.59]
RankingsWhere HBE1 stands among ~20K protein-coding genes
  • #4,403of 20,598
    Most Researched108 · top quartile
  • #15,643of 17,882
    Most Constrained (LOEUF)1.59
Genes detectedHBE1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
εγ-Thalassemia, a New Hemoglobinopathy Category.
PMID: 37086467
Clin Chem · 2023
1.00
2
Predictive SNPs for β
PMID: 33990643
Sci Rep · 2021
0.90
3
Comprehensive Characterization and Global Transcriptome Analysis of Human Fetal Liver Terminal Erythropoiesis.
PMID: 37657739
Genomics Proteomics Bioinformatics · 2023
0.80
4
Characterization of human mucin 5B gene expression in airway epithelium and the genomic clone of the amino-terminal and 5'-flanking region.
PMID: 11713095
Am J Respir Cell Mol Biol · 2001
0.70
5
Identifying signatures of positive selection in human populations from North Africa.
PMID: 37210386
Sci Rep · 2023
0.60