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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GABARAPL2
GABA type A receptor associated protein like 2
Chromosome 16 · 16q23.1
NCBI Gene: 11345Ensembl: ENSG00000034713.8HGNC: HGNC:13291UniProt: P60520
156PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneTransporter
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
phosphatidylethanolamine bindingubiquitin protein ligase bindingGolgi membraneprotein bindingsmoking initiationattention deficit hyperactivity disordersubstance abuseextrapyramidal and movement disease
✦AI Summary

GABARAPL2 (GABA type A receptor associated protein like 2) is a ubiquitin-like modifier protein that plays critical roles in both autophagy and intracellular membrane trafficking. The protein functions as an essential component of the autophagy machinery, particularly in the maturation stages of autophagosome formation, where it works alongside other LC3/GABARAP family members to facilitate cargo recognition and vesicle closure 1. GABARAPL2 is involved in selective autophagy processes, including mitophagy for mitochondrial quality control and ferritinophagy for iron homeostasis regulation 23. The protein contains LC3-interacting region (LIR) motifs that enable specific cargo receptor interactions and targeting to autophagosomes 4. Beyond autophagy, GABARAPL2 modulates intra-Golgi transport by coupling NSF ATPase activity with SNARE protein activation. Recent studies have identified GABARAPL2 as a binding partner for IRGQ in MHC class I quality control, where it directs misfolded MHC molecules toward lysosomal degradation, potentially contributing to tumor immune evasion mechanisms 5. The protein shows altered expression in various disease contexts, including diabetes mellitus and pulmonary fibrosis, where it regulates autophagy-dependent cellular processes 67. These diverse functions establish GABARAPL2 as a crucial regulator of cellular homeostasis through both autophagy-dependent and independent mechanisms.

Sources cited
1
GABARAPL2 is part of the LC3/GABARAP family involved in autophagosome maturation and cargo recognition
PMID: 27601442
2
GABARAPL2 serves as an autophagy marker and is involved in ammonia-induced autophagy and mitophagy
PMID: 25700560
3
GABARAPL2 is enriched in autophagosomes and involved in selective autophagy processes like ferritinophagy
PMID: 24695223
4
GABARAPL2 contains LIR motifs crucial for selective autophagy and cargo receptor interactions
PMID: 23908376
5
GABARAPL2 binds to IRGQ in MHC class I quality control and directs misfolded MHC molecules to lysosomal degradation
PMID: 39481378
6
GABARAPL2 shows altered expression in type 2 diabetes mellitus as a hub autophagy-related gene
PMID: 37223013
7
GABARAPL2 is involved in autophagy regulation in pulmonary fibrosis context
PMID: 35427207
Disease Associationsⓘ20
smoking initiationOpen Targets
0.07Suggestive
attention deficit hyperactivity disorderOpen Targets
0.07Suggestive
substance abuseOpen Targets
0.07Suggestive
extrapyramidal and movement diseaseOpen Targets
0.06Suggestive
Blackfan-Diamond anemiaOpen Targets
0.05Suggestive
hemoglobin D diseaseOpen Targets
0.05Suggestive
dominant beta-thalassemiaOpen Targets
0.04Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.04Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.04Suggestive
Alpha-thalassemia - myelodysplastic syndromeOpen Targets
0.04Suggestive
alpha-thalassemia-myelodysplastic syndromeOpen Targets
0.04Suggestive
X-linked severe congenital neutropeniaOpen Targets
0.04Suggestive
immunoglobulin-mediated membranoproliferative glomerulonephritisOpen Targets
0.04Suggestive
nephrotic syndromeOpen Targets
0.04Suggestive
hemoglobin H diseaseOpen Targets
0.04Suggestive
congenital neutropenia-myelofibrosis-nephromegaly syndromeOpen Targets
0.04Suggestive
hemoglobin E diseaseOpen Targets
0.04Suggestive
Recurrent infections-myelofibrosis-nephromegaly syndromeOpen Targets
0.04Suggestive
neutropenia, severe congenital, 2, autosomal dominantOpen Targets
0.04Suggestive
neutropenia, severe congenital, 10, autosomal recessiveOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATG16L1Protein interaction100%BNIP3LProtein interaction100%BNIP3Protein interaction100%UBA5Protein interaction99%ATG4DProtein interaction99%ATG4CProtein interaction99%
Tissue Expression6 tissues
Brain
100%
Heart
91%
Lung
40%
Bone Marrow
37%
Ovary
37%
Liver
28%
Gene Interaction Network
Click a node to explore
GABARAPL2ATG16L1BNIP3LBNIP3UBA5ATG4DATG4C
PROTEIN STRUCTURE
Preparing viewer…
PDB7YO8 · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.58LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.89 [0.52–1.58]
RankingsWhere GABARAPL2 stands among ~20K protein-coding genes
  • #2,886of 20,598
    Most Researched156 · top quartile
  • #15,589of 17,882
    Most Constrained (LOEUF)1.58
Genes detectedGABARAPL2
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
SIRT5 regulation of ammonia-induced autophagy and mitophagy.
PMID: 25700560
Autophagy · 2015
1.00
2
LC3/GABARAP family proteins: autophagy-(un)related functions.
PMID: 27601442
FASEB J · 2016
0.90
3
Autophagy mediates degradation of nuclear lamina.
PMID: 26524528
Nature · 2015
0.80
4
Quantitative proteomics identifies NCOA4 as the cargo receptor mediating ferritinophagy.
PMID: 24695223
Nature · 2014
0.70
5
Live imaging of intra-lysosome pH in cell lines and primary neuronal culture using a novel genetically encoded biosensor.
PMID: 32515674
Autophagy · 2021
0.60