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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GABARAP
GABA type A receptor-associated protein
Chromosome 17 · 17p13.1
NCBI Gene: 11337Ensembl: ENSG00000170296.11HGNC: HGNC:4067UniProt: O95166
179PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneTransporter
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingphosphatidylethanolamine bindingubiquitin protein ligase bindingbeta-tubulin bindingneurodegenerative diseasebreast cancerosteosarcomaneoplasm
✦AI Summary

GABARAP (GABA type A receptor-associated protein) is a ubiquitin-like protein with dual roles in intracellular transport and selective autophagy. As an autophagy-related protein, GABARAP functions distinctly from LC3 family members: while LC3s promote phagophore elongation, the GABARAP/GATE-16 subfamily is essential for later-stage autophagosome maturation 1. GABARAP plays critical roles in specialized autophagic pathways including reticulophagy, where it interacts with ER-phagy receptor TEX264 to mediate endoplasmic reticulum remodeling and turnover under nutrient stress 2, and in mitophagy, where mitochondrial cargo receptors target dysfunctional mitochondria to autophagosomes via GABARAP interaction 3. Beyond autophagy, GABARAP regulates cytoskeletal organization and cell migration through its interaction with the CUL3(KBTBD6/7) E3 ubiquitin ligase complex, which ubiquitinates the RAC1-activating GEF TIAM1 4. Recent evidence demonstrates GABARAP's role in immune responses: upon cGAS-STING pathway activation, GABARAP lipidation on STING vesicles sequesters the FLCN-FNIP complex, preventing mTORC1-mediated phosphorylation of transcription factor TFEB and promoting lysosome biogenesis for enhanced pathogen clearance 5. Dysregulation of GABARAP-dependent autophagy is implicated in fatty liver disease, where impaired lipophagy contributes to disease pathogenesis 6.

Sources cited
1
GABARAP subfamily is essential for later-stage autophagosome maturation, distinct from LC3s which promote phagophore elongation
PMID: 27601442
2
GABARAP interacts with ER-phagy receptor TEX264 to mediate endoplasmic reticulum autophagy under nutrient stress
PMID: 31006538
3
Mitochondrial cargo receptors target dysfunctional mitochondria to autophagosomes through interaction with LC3/GABARAP family members
PMID: 33580835
4
GABARAP regulates CUL3(KBTBD6/7) E3 ubiquitin ligase complex that ubiquitinates TIAM1, affecting cytoskeletal organization and cell migration
PMID: 25684205
5
GABARAP lipidation upon cGAS-STING activation sequesters FLCN-FNIP complex to promote TFEB-mediated lysosome biogenesis and pathogen clearance
PMID: 39689715
6
Impaired GABARAP-dependent lipophagy contributes to fatty liver disease pathogenesis
PMID: 33794741
7
GABARAP serves as an autophagy marker used to measure ammonia-induced autophagy and mitophagy
PMID: 25700560
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.46Moderate
breast cancerOpen Targets
0.09Suggestive
osteosarcomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
SepsisOpen Targets
0.04Suggestive
infectionOpen Targets
0.04Suggestive
coronary artery diseaseOpen Targets
0.03Suggestive
nontoxic goiterOpen Targets
0.03Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.03Suggestive
age-related hearing impairmentOpen Targets
0.03Suggestive
hypertensionOpen Targets
0.02Suggestive
hypothyroidismOpen Targets
0.02Suggestive
pancreatic adenocarcinomaOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.02Suggestive
hearing lossOpen Targets
0.02Suggestive
Alzheimer diseaseOpen Targets
0.02Suggestive
Lewy body dementiaOpen Targets
0.02Suggestive
obesityOpen Targets
0.02Suggestive
type 1 diabetes mellitusOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ATG16L1Protein interaction100%ATG14Protein interaction100%SEC62Protein interaction100%ATG4AProtein interaction100%ATG4DProtein interaction99%ATG4CProtein interaction99%
Tissue Expression6 tissues
Brain
100%
Heart
42%
Lung
37%
Ovary
36%
Liver
26%
Bone Marrow
16%
Gene Interaction Network
Click a node to explore
GABARAPATG16L1ATG14SEC62ATG4AATG4DATG4C
PROTEIN STRUCTURE
Preparing viewer…
PDB6HYO · 1.07 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.44LoF Tolerant
pLIⓘ
0.23Tolerant
Observed/Expected LoF0.48 [0.19–1.44]
RankingsWhere GABARAP stands among ~20K protein-coding genes
  • #2,434of 20,598
    Most Researched179 · top quartile
  • #14,786of 17,882
    Most Constrained (LOEUF)1.44
Genes detectedGABARAP
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
SIRT5 regulation of ammonia-induced autophagy and mitophagy.
PMID: 25700560
Autophagy · 2015
1.00
2
The cGAS-STING pathway activates transcription factor TFEB to stimulate lysosome biogenesis and pathogen clearance.
PMID: 39689715
Immunity · 2025
0.90
3
LC3/GABARAP family proteins: autophagy-(un)related functions.
PMID: 27601442
FASEB J · 2016
0.80
4
The ménage à trois of autophagy, lipid droplets and liver disease.
PMID: 33794741
Autophagy · 2022
0.70
5
CCT2 is an aggrephagy receptor for clearance of solid protein aggregates.
PMID: 35366418
Cell · 2022
0.60