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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PLEKHA1
pleckstrin homology domain containing A1
Chromosome 10 · 10q26.13
NCBI Gene: 59338Ensembl: ENSG00000107679.15HGNC: HGNC:14335UniProt: B3KQL5
69PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingplasma membranenucleoplasmcytosoltype 2 diabetes mellitushypothyroidismmacular degenerationthyroid disease
✦AI Summary

PLEKHA1 (pleckstrin homology domain containing A1) is a phosphoinositide-binding protein that specifically recognizes phosphatidylinositol 3,4-diphosphate and recruits proteins to the plasma membrane, functioning in PI3K/AKT signaling regulation. The protein is involved in cellular responses including ruffle organization and B cell receptor signaling. Mechanistically, PLEKHA1 contains a pleckstrin homology domain enabling phospholipid binding and PDZ domain interactions for protein localization 1. Clinically, PLEKHA1 variants show cumulative association with age-related macular degeneration (AMD) risk across multiple ethnic populations 2 3. In Alzheimer's disease (AD), PLEKHA1 expression in specific immune cell types is causally associated with disease risk 4, with tandem repeat variation in PLEKHA1 linked to AD pathogenesis 5. A genomewide analysis identified PLEKHA1 in microglial and oligodendrocytic gene networks altered during aging and contributing to AD susceptibility 6. In kidney disease, PLEKHA1 is dysregulated in proximal tubule cells and functions as a disease marker; knockdown increases PT differentiation gene expression, suggesting pathogenic involvement 1. In esophageal cancer, oncogenic PLEKHA1-TACC2 fusion proteins drive tumorigenesis via EphA2/AKT/MMP2 pathway activation and vascular mimicry formation 7. PLEKHA1 also serves as a shared diagnostic biomarker in systemic lupus erythematosus and ulcerative colitis, regulating neutrophil activation and neutrophil extracellular trap formation 8.

Sources cited
1
PLEKHA1 identified as disease marker in kidney disease; knockdown increases PT differentiation gene expression
PMID: 38513647
2
Genetic variants in PLEKHA1/ARMS2/HTRA1 cluster show cumulative association with AMD across ethnic groups
PMID: 24013816
3
PLEKHA1 associated with age-related maculopathy susceptibility
PMID: 17000705
4
PLEKHA1 expression in immune cells causally associated with Alzheimer's disease risk
PMID: 39455528
5
Tandem repeat variation in PLEKHA1 associated with Alzheimer's disease
PMID: 39809899
6
PLEKHA1 identified in microglial and oligodendrocytic networks altered with aging and AD pathogenesis
PMID: 39778705
7
PLEKHA1-TACC2 fusion protein drives tumorigenesis via EphA2/AKT/MMP2 pathway in esophageal squamous cell carcinoma
PMID: 40615663
8
PLEKHA1 serves as shared diagnostic biomarker in SLE and UC; regulates neutrophil activation and NET formation
PMID: 40379887
Disease Associationsⓘ20
type 2 diabetes mellitusOpen Targets
0.50Moderate
hypothyroidismOpen Targets
0.48Moderate
macular degenerationOpen Targets
0.43Moderate
thyroid diseaseOpen Targets
0.40Moderate
diabetes mellitusOpen Targets
0.40Moderate
HeadacheOpen Targets
0.38Weak
Ischemic strokeOpen Targets
0.34Weak
diverticular diseaseOpen Targets
0.32Weak
head and neck malignant neoplasiaOpen Targets
0.32Weak
Hashimoto's thyroiditisOpen Targets
0.32Weak
autoimmune diseaseOpen Targets
0.32Weak
essential hypertensionOpen Targets
0.30Weak
neurodegenerative diseaseOpen Targets
0.30Weak
autoimmune thyroid diseaseOpen Targets
0.30Weak
intelligenceOpen Targets
0.29Weak
schizophreniaOpen Targets
0.29Weak
attention deficit hyperactivity disorderOpen Targets
0.28Weak
PainOpen Targets
0.27Weak
migraine disorderOpen Targets
0.26Weak
degeneration of macula and posterior poleOpen Targets
0.26Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PTPN13Protein interaction97%ARMS2Protein interaction95%CFHProtein interaction85%HTRA1Protein interaction85%PLEKHA2Protein interaction74%BMXShared pathway18%
Tissue Expression6 tissues
Brain
100%
Heart
99%
Liver
89%
Lung
60%
Ovary
57%
Bone Marrow
22%
Gene Interaction Network
Click a node to explore
PLEKHA1PTPN13ARMS2CFHHTRA1PLEKHA2BMX
PROTEIN STRUCTURE
Preparing viewer…
PDB1EAZ · 1.40 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.96LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.69 [0.51–0.96]
RankingsWhere PLEKHA1 stands among ~20K protein-coding genes
  • #6,830of 20,598
    Most Researched69
  • #8,982of 17,882
    Most Constrained (LOEUF)0.96
Genes detectedPLEKHA1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Transcriptomic, epigenomic, and spatial metabolomic cell profiling redefines regional human kidney anatomy.
PMID: 38513647
Cell Metab · 2024
1.00
2
Cumulative association between age-related macular degeneration and less studied genetic variants in PLEKHA1/ARMS2/HTRA1: a meta and gene-cluster analysis.
PMID: 24013816
Mol Biol Rep · 2013
0.90
3
The PLEKHA1-TACC2 fusion gene drives tumorigenesis via vascular mimicry formation in esophageal squamous-cell carcinoma.
PMID: 40615663
Cell Death Differ · 2025
0.80
4
Identification of JAZF1, KNOP1, and PLEKHA1 as causally associated genes and drug targets for Alzheimer's disease: a summary data-based Mendelian randomization study.
PMID: 39455528
Inflammopharmacology · 2024
0.70
5
Human longevity and Alzheimer's disease variants act via microglia and oligodendrocyte gene networks.
PMID: 39778705
Brain · 2025
0.60