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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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NAAA
N-acylethanolamine acid amidase
Chromosome 4 · 4q21.1
NCBI Gene: 27163Ensembl: ENSG00000138744.18HGNC: HGNC:736UniProt: Q02083
30PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
hydrolase activity, acting on carbon-nitrogen (but not peptide) bondsN-acylsphingosine amidohydrolase activityfatty acid amide hydrolase activityN-(long-chain-acyl)ethanolamine deacylase activityabscesscellulitisTietze syndromefamilial hemolytic anemia
✦AI Summary

NAAA (N-acylethanolamine acid amidase) is a lysosomal cysteine hydrolase that degrades bioactive fatty acid amides, with the highest activity toward palmitoylethanolamide (PEA) followed by other N-acylethanolamines 1. The enzyme exhibits optimal catalytic activity at acidic pH (4.5-5.0), consistent with its lysosomal localization 1. NAAA is highly expressed in immune tissues including lung, spleen, thymus, and particularly in alveolar macrophages 1. Mechanistically, NAAA controls inflammation by hydrolyzing PEA, an anti-inflammatory PPAR-α agonist, thereby regulating PPAR-α signaling pathways 2. This regulation appears critical for both induction and resolution of inflammatory responses 2. NAAA demonstrates significant disease relevance across multiple pathological conditions. In inflammatory bowel disease, increased NAAA expression correlates with intestinal fibrosis, and NAAA inhibition reduces fibrosis through modulation of macrophage activity and IL-23 signaling 3. In pain chr4, NAAA activation in monocytes enables hyperalgesic priming through suppression of PPAR-α signaling 4. Additionally, NAAA contributes to neurodegeneration, with elevated expression observed in Parkinson's disease patients and mouse models, where NAAA inhibition protects dopamine neurons 5. These findings establish NAAA as a promising therapeutic target for anti-inflammatory and neuroprotective interventions.

Sources cited
1
NAAA is a lysosomal cysteine hydrolase with acidic pH optimum and high expression in immune tissues
PMID: 17712833
2
NAAA controls inflammation by regulating PEA-PPAR-α signaling pathways
PMID: 32191459
3
NAAA expression is increased in intestinal fibrosis and its inhibition reduces fibrosis through macrophage modulation
PMID: 39211986
4
NAAA activation in monocytes enables pain chronification through PPAR-α suppression
PMID: 38402219
5
NAAA is elevated in Parkinson's disease and its inhibition protects dopamine neurons
PMID: 35781057
Disease Associationsⓘ20
abscessOpen Targets
0.27Weak
cellulitisOpen Targets
0.27Weak
Tietze syndromeOpen Targets
0.27Weak
familial hemolytic anemiaOpen Targets
0.20Weak
Blackfan-Diamond anemiaOpen Targets
0.07Suggestive
Adult-onset autosomal recessive sideroblastic anemiaOpen Targets
0.06Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.06Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.06Suggestive
inosine triphosphatase deficiencyOpen Targets
0.06Suggestive
hereditary persistence of fetal hemoglobin-sickle cell disease syndromeOpen Targets
0.06Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.06Suggestive
primary familial polycythemia due to EPO receptor mutationOpen Targets
0.06Suggestive
microcytic anemia with liver iron overloadOpen Targets
0.06Suggestive
Alpha-thalassemia - myelodysplastic syndromeOpen Targets
0.05Suggestive
alpha-thalassemia-myelodysplastic syndromeOpen Targets
0.05Suggestive
hemophagocytic syndromeOpen Targets
0.05Suggestive
hemoglobin D diseaseOpen Targets
0.05Suggestive
alpha thalassemia-intellectual disability syndrome type 1Open Targets
0.05Suggestive
Alpha-thalassemia - intellectual disability syndrome linked to chromosome 16Open Targets
0.05Suggestive
IRIDA syndromeOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VAC14Protein interaction100%MGLLProtein interaction97%FAAHProtein interaction85%LAMP2Protein interaction84%FIG4Protein interaction84%FAAH2Protein interaction75%
Tissue Expression6 tissues
Liver
100%
Lung
51%
Bone Marrow
49%
Heart
47%
Brain
23%
Ovary
21%
Gene Interaction Network
Click a node to explore
NAAAVAC14MGLLFAAHLAMP2FIG4FAAH2
PROTEIN STRUCTURE
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PDB6DXW · 2.30 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.14LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.65–1.14]
RankingsWhere NAAA stands among ~20K protein-coding genes
  • #11,979of 20,598
    Most Researched30
  • #11,832of 17,882
    Most Constrained (LOEUF)1.14
Genes detectedNAAA
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PMID: 32191459
J Med Chem · 2020
1.00
2
Pharmacological Inhibition of N-Acylethanolamine Acid Amidase (NAAA) Mitigates Intestinal Fibrosis Through Modulation of Macrophage Activity.
PMID: 39211986
J Crohns Colitis · 2025
0.90
3
NAAA-regulated lipid signaling in monocytes controls the induction of hyperalgesic priming in mice.
PMID: 38402219
Nat Commun · 2024
0.80
4
The N-acylethanolamine-hydrolyzing acid amidase (NAAA).
PMID: 17712833
Chem Biodivers · 2007
0.70
5
Polyethylene terephthalate microplastics exposure enhances the risk of ulcerative colitis: insights from multiomics integration, machine learning, and molecular docking reveal intestinal toxicity mechanisms.
PMID: 40981471
Int J Surg · 2026
0.60