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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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WIPI2
WD repeat domain, phosphoinositide interacting 2
Chromosome 7 Β· 7p22.1
NCBI Gene: 26100Ensembl: ENSG00000157954.16HGNC: HGNC:32225UniProt: Q9Y4P8
79PubMed Papers
21Diseases
0Drugs
2Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein-containing complexautophagosome maturationmembraneprotein bindingintellectual developmental disorder with short stature and variable skeletal anomaliesneurodegenerative diseaseautosomal recessive non-syndromic intellectual disabilitysmoking initiation
✦AI Summary

WIPI2 (WD repeat domain, phosphoinositide interacting 2) is a critical autophagy regulator that recruits the ATG12-ATG5-ATG16L1 complex to omegasomes and preautophagosomal structures, initiating ATG8 lipidation and autophagosome formation 1. The protein binds phosphatidylinositol-3-phosphate (PI3P) and functions in starvation-induced autophagy and selective autophagy of pathogenic bacteria, including Salmonella 1. WIPI2 serves as a key component in the cGAS-STING innate immune pathway, where it directly interacts with STING to enable LC3 lipidation independent of canonical VPS34-beclin complexes, facilitating cytoplasmic DNA and viral clearance 23. During mitosis, WIPI2 is polyubiquitinated and degraded via CRL4 ubiquitin ligases, suppressing autophagy during cell division 1. WIPI2 is also recruited to endoplasmic reticulum subdomains by VAPs (VAMP-associated proteins) to facilitate reticulophagy, the selective autophagy of impaired ER regions 4. Disease associations include Crohn's disease, age-related macular degeneration, hepatocellular carcinoma, and colorectal cancer, reflecting autophagy's role in cellular homeostasis and immune function. WIPI2 represents a therapeutic target for autophagy-related disorders and conditions benefiting from enhanced selective autophagy.

Sources cited
1
WIPI2 recruits ATG12-ATG5-ATG16L1 complex and is degraded during mitosis via CRL4-mediated polyubiquitination
PMID: 30898011
2
WIPI2 is required for cGAMP-STING-induced LC3 lipidation independent of ULK and VPS34-beclin complexes
PMID: 30842662
3
STING directly interacts with WIPI2's PI3P-binding motif to initiate autophagosome formation and compete with canonical PI3P-dependent autophagy
PMID: 36872914
4
WIPI2 is recruited to ER subdomains by VAPs to facilitate RETREG1-mediated reticulophagy during nutrient deprivation
PMID: 38705724
5
WIPI2 is a core autophagy component whose regulation involves post-translational modifications including acetylation
PMID: 35435793
6
WIPI2 participates in autophagosome assembly and is relevant to endometrial and cancer biology
PMID: 32401642
Disease Associationsβ“˜21
intellectual developmental disorder with short stature and variable skeletal anomaliesOpen Targets
0.55Moderate
neurodegenerative diseaseOpen Targets
0.54Moderate
autosomal recessive non-syndromic intellectual disabilityOpen Targets
0.46Moderate
smoking initiationOpen Targets
0.40Weak
lysosomal storage diseaseOpen Targets
0.37Weak
breast carcinomaOpen Targets
0.36Weak
post term pregnancyOpen Targets
0.22Weak
facial nerve diseaseOpen Targets
0.09Suggestive
age-related macular degenerationOpen Targets
0.08Suggestive
X-linked retinal dysplasiaOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
colorectal cancerOpen Targets
0.07Suggestive
retinitis pigmentosaOpen Targets
0.07Suggestive
colorectal carcinomaOpen Targets
0.07Suggestive
choroidal dystrophy, central areolar, 1Open Targets
0.06Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.06Suggestive
Crohn's diseaseOpen Targets
0.06Suggestive
severe early-childhood-onset retinal dystrophyOpen Targets
0.06Suggestive
Stargardt diseaseOpen Targets
0.06Suggestive
reticular dystrophy of the retinal pigment epitheliumOpen Targets
0.06Suggestive
Intellectual developmental disorder with short stature and variable skeletal anomaliesUniProt
Pathogenic Variants2
NM_015610.4(WIPI2):c.551T>G (p.Val184Gly)Pathogenic
Intellectual developmental disorder with short stature and variable skeletal anomalies
β˜†β˜†β˜†β˜†2021β†’ Residue 184
NM_015610.4(WIPI2):c.745G>A (p.Val249Met)Pathogenic
Intellectual developmental disorder with short stature and variable skeletal anomalies
β˜†β˜†β˜†β˜†2021β†’ Residue 249
View on ClinVar β†—
Related Genes
BECN1Protein interaction100%ATG16L1Protein interaction100%ATG101Protein interaction100%PIK3R4Protein interaction99%TECPR1Protein interaction98%OTUD7BProtein interaction96%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
68%
Liver
66%
Brain
62%
Lung
60%
Ovary
58%
Gene Interaction Network
Click a node to explore
WIPI2BECN1ATG16L1ATG101PIK3R4TECPR1OTUD7B
PROTEIN STRUCTURE
Preparing viewer…
PDB7F69 Β· 1.50 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.77LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.56 [0.41–0.77]
RankingsWhere WIPI2 stands among ~20K protein-coding genes
  • #6,045of 20,598
    Most Researched79
  • #4,361of 5,498
    Most Pathogenic Variants2
  • #6,238of 17,882
    Most Constrained (LOEUF)0.77
Genes detectedWIPI2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Acetylation in the regulation of autophagy.
PMID: 35435793
Autophagy Β· 2023
1.00
2
Autophagy induction via STING trafficking is a primordial function of the cGAS pathway.
PMID: 30842662
Nature Β· 2019
0.90
3
Autophagy in the physiological endometrium and cancer.
PMID: 32401642
Autophagy Β· 2021
0.80
4
USP20 deubiquitinates and stabilizes the reticulophagy receptor RETREG1/FAM134B to drive reticulophagy.
PMID: 38705724
Autophagy Β· 2024
0.70
5
Suppression of autophagy during mitosis via CUL4-RING ubiquitin ligases-mediated WIPI2 polyubiquitination and proteasomal degradation.
PMID: 30898011
Autophagy Β· 2019
0.60