HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
3 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
HHLA1
HHLA1 neighbor of OC90
Chromosome 8 · 8q24.22
NCBI Gene: 10086Ensembl: ENSG00000132297.13HGNC: HGNC:4904UniProt: C9JL84
6PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Swiss-Prot Reviewed
extracellular regionRespiratory insufficiencyneurodegenerative diseasepernicious anemiapolycythemia
✦AI Summary

HHLA1 (HERV-H LTR-associating 1) is a gene of unknown primary function located on chromosome 8, named for its association with a human endogenous retrovirus HERV-H long terminal repeat (LTR) element 1. The gene exhibits sequence conservation across mammalian species, suggesting evolutionary importance 1. HHLA1 is transcriptionally regulated by the HERV-H LTR promoter and can undergo intergenic splicing with the adjacent downstream gene OC90 (otoconin-90) in certain cell contexts, particularly teratocarcinoma cell lines, resulting in fusion transcripts 1. Mechanistically, HHLA1 shows functional association with HHLA2 and MAGEB5 proteins in relation to HLA class I and II expression modulation, with potential involvement in viral-related pathways 2. Disease relevance includes potential connections to virus-related tumors and oncogenic diseases, where HHLA2 and MAGEB5 co-expression with HHLA1 may serve as disease markers 2. However, the specific molecular function of HHLA1 itself remains undefined, and direct clinical applications require further investigation. The gene's precise role in normal physiology and disease pathogenesis warrants additional functional studies.

Sources cited
1
HHLA1 is transcribed from HERV-H LTR promoter, shows mammalian sequence conservation, and undergoes intergenic splicing with OC90 in teratocarcinoma cells
PMID: 10329003
2
HHLA1 shows functional association with HHLA2 and MAGEB5 in relation to HLA expression changes and potential viral-related tumor markers
PMID: 34320928
⚠Limited data available — This gene has 2 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
Respiratory insufficiencyOpen Targets
0.30Weak
neurodegenerative diseaseOpen Targets
0.21Weak
pernicious anemiaOpen Targets
0.17Weak
polycythemiaOpen Targets
0.11Weak
hypothyroidismOpen Targets
0.07Suggestive
breast carcinomaOpen Targets
0.04Suggestive
polyarteritis nodosaOpen Targets
0.03Suggestive
rosaceaOpen Targets
0.03Suggestive
ovarian neoplasmOpen Targets
0.02Suggestive
drug allergyOpen Targets
0.02Suggestive
hypertrophic cardiomyopathyOpen Targets
0.02Suggestive
ataxia telangiectasiaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
teratocarcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Klinefelter's syndromeOpen Targets
0.00Suggestive
metastatic neoplasmOpen Targets
0.00Suggestive
ovarian cancerOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
sarcomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
OC90Protein interaction97%
Tissue Expression6 tissues
Brain
100%
Lung
30%
Liver
28%
Ovary
18%
Bone Marrow
0%
Heart
0%
Gene Interaction Network
Click a node to explore
HHLA1OC90
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt C9JL84
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.25LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.96 [0.74–1.25]
RankingsWhere HHLA1 stands among ~20K protein-coding genes
  • #18,093of 20,598
    Most Researched6
  • #13,170of 17,882
    Most Constrained (LOEUF)1.25
Genes detectedHHLA1
Sources retrieved3 papers
Response time—
📄 Sources
3
1
Targeted immune epitope prediction to HHLA2 and MAGEB5 protein variants as therapeutic approach to related viral diseases.
PMID: 34320928
BMC Immunol · 2021
1.00
2
Intergenic splicing between a HERV-H endogenous retrovirus and two adjacent human genes.
PMID: 10329003
Genomics · 1999
0.67
3
Whole exome sequencing, in silico and functional studies confirm the association of the
PMID: 34354426
Saudi J Biol Sci · 2021
0.33