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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ATP8B4
ATPase phospholipid transporting 8B4 (putative)
Chromosome 15 · 15q21.2
NCBI Gene: 79895Ensembl: ENSG00000104043.16HGNC: HGNC:13536UniProt: Q6PG43
19PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
Golgi apparatusplasma membraneprotein bindingphospholipid translocationAlzheimer diseasebasal cell carcinomabenign neoplasm of eyeVitiligo
✦AI Summary

ATP8B4 is a putative P4-ATPase flippase that catalyzes ATP hydrolysis coupled to aminophospholipid transport from the outer to inner leaflet of cellular membranes, maintaining asymmetric phospholipid distribution and facilitating vesicle formation. Recent genetic studies have identified ATP8B4 as a significant disease risk factor across multiple conditions. Rare damaging variants in ATP8B4 are associated with increased Alzheimer's disease risk, particularly in early-onset cases 1. A missense variant (F436L) significantly increases systemic sclerosis risk, with differential ATP8B4 expression observed in affected patients 2. The gene also shows mechanosensitive upregulation in cardiac fibroblasts under varying matrix stiffness conditions, suggesting roles in cardiac fibrosis pathogenesis 3. Additionally, ATP8B4 variants appear enriched among deleterious mutations in populations and may contribute to HIV-1 susceptibility through glycolysis/gluconeogenesis pathway modulation 4. ATP8B4 variants are now included in clinical genetic screening panels for Alzheimer's disease, representing one of the rare risk factors with moderate-to-strong effects 5. These findings collectively highlight ATP8B4's emerging importance in multiple disease contexts beyond its fundamental role in membrane phospholipid homeostasis.

Sources cited
1
Rare damaging variants in ATP8B4 are associated with increased Alzheimer's disease risk
PMID: 36411364
2
A missense variant (F436L) significantly increases systemic sclerosis risk, with differential ATP8B4 expression in patients
PMID: 26473621
3
ATP8B4 shows mechanosensitive upregulation in cardiac fibroblasts and is involved in cardiac fibrosis
PMID: 40830643
4
ATP8B4 variants are enriched among deleterious mutations and may contribute to HIV-1 susceptibility
PMID: 38179408
5
ATP8B4 is included in clinical genetic screening panels for Alzheimer's disease as a rare risk factor
PMID: 38281098
Disease Associationsⓘ20
Alzheimer diseaseOpen Targets
0.43Moderate
basal cell carcinomaOpen Targets
0.42Moderate
benign neoplasm of eyeOpen Targets
0.38Weak
VitiligoOpen Targets
0.31Weak
agingOpen Targets
0.31Weak
lobe attachmentOpen Targets
0.29Weak
lymphatic system diseaseOpen Targets
0.29Weak
abscessOpen Targets
0.28Weak
cellulitisOpen Targets
0.28Weak
ovarian neoplasmOpen Targets
0.27Weak
bipolar disorderOpen Targets
0.26Weak
rhabdomyolysisOpen Targets
0.25Weak
cardiomyopathyOpen Targets
0.23Weak
liver diseaseOpen Targets
0.05Suggestive
colorectal carcinomaOpen Targets
0.04Suggestive
vascular diseaseOpen Targets
0.04Suggestive
intestinal diseaseOpen Targets
0.04Suggestive
KeloidOpen Targets
0.03Suggestive
bone fractureOpen Targets
0.03Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATP8B3Shared pathway100%CDC50AProtein interaction94%ATP8B2Shared pathway75%ATP10BShared pathway50%ATP11BShared pathway40%ATP9BShared pathway40%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
3%
Lung
3%
Ovary
3%
Heart
2%
Brain
1%
Gene Interaction Network
Click a node to explore
ATP8B4ATP8B3CDC50AATP8B2ATP10BATP11BATP9B
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q6PG43
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.01LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.72–1.01]
RankingsWhere ATP8B4 stands among ~20K protein-coding genes
  • #14,362of 20,598
    Most Researched19
  • #9,893of 17,882
    Most Constrained (LOEUF)1.01
Genes detectedATP8B4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
An integrated machine learning framework for developing and validating a diagnostic model of major depressive disorder based on interstitial cystitis-related genes.
PMID: 38754597
J Affect Disord · 2024
1.00
2
Exome sequencing identifies rare damaging variants in ATP8B4 and ABCA1 as risk factors for Alzheimer's disease.
PMID: 36411364
Nat Genet · 2022
0.90
3
Decoding mechanosensitive genes in cardiac fibroblasts via 3D hydrogel models of fibrosis.
PMID: 40830643
Sci Rep · 2025
0.80
4
Lessons from genetic studies in Alzheimer disease.
PMID: 38429159
Rev Neurol (Paris) · 2024
0.70
5
Whole genome sequencing reveals population diversity and variation in HIV-1 specific host genes.
PMID: 38179408
Front Genet · 2023
0.60