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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ZDHHC13
zDHHC palmitoyltransferase 13
Chromosome 11 · 11p15.1
NCBI Gene: 54503Ensembl: ENSG00000177054.15HGNC: HGNC:18413UniProt: Q8IUH4
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
membranepositive regulation of canonical NF-kappaB signal transductionendoplasmic reticulumGolgi membranerectosigmoid junction neoplasmneurodegenerative diseaseAlopecia-intellectual disability syndromeAlopecia universalis
✦AI Summary

ZDHHC13 encodes a palmitoyltransferase that catalyzes the addition of palmitate groups to various protein substrates, playing critical roles in cellular signaling and disease processes 1. The enzyme palmitoylates key proteins including pyruvate kinase M2 (PKM2), where palmitoylation at Cys31 impairs PKM2 tetramerization and glycolysis, contributing to endothelial injury and cardiovascular dysfunction 1. ZDHHC13 is essential for melanocortin-1 receptor (MC1R) palmitoylation, which activates MC1R signaling, enhances DNA repair after UV irradiation, and prevents melanomagenesis 2. AMPK phosphorylation of ZDHHC13 at S208 strengthens its interaction with MC1R variants, providing a potential therapeutic target for melanoma prevention in red-haired individuals 3. The enzyme also palmitoylates ULK1, facilitating its translocation to autophagosome formation sites and initiating autophagy 4. Loss-of-function mutations in ZDHHC13 cause severe phenotypes in mice including alopecia, osteoporosis, and systemic amyloidosis, establishing palmitoylation as crucial for diverse physiological functions 5. Clinical relevance extends to neurological diseases, with ZDHHC13 expression changes associated with Parkinson's disease progression through DNA methylation regulation 6 and protective effects against ischemic stroke through metabolite regulation 7.

Sources cited
1
ZDHHC13 palmitoylates PKM2 at Cys31, contributing to endothelial injury and cardiovascular dysfunction
PMID: 39665133
2
ZDHHC13-mediated MC1R palmitoylation is essential for MC1R signaling and melanoma prevention
PMID: 28869973
3
AMPK phosphorylates ZDHHC13 at S208 to enhance MC1R-RHC interaction and suppress melanomagenesis
PMID: 36701140
4
ZDHHC13 palmitoylates ULK1 for autophagosome formation and autophagy initiation
PMID: 39169022
5
Loss-of-function ZDHHC13 mutations cause alopecia, osteoporosis, and systemic amyloidosis in mice
PMID: 20548961
6
ZDHHC13 expression changes are associated with Parkinson's disease progression
PMID: 40088380
7
ZDHHC13 provides protective effects against ischemic stroke through metabolite regulation
PMID: 40711616
Disease Associationsⓘ20
rectosigmoid junction neoplasmOpen Targets
0.25Weak
neurodegenerative diseaseOpen Targets
0.21Weak
Alopecia-intellectual disability syndromeOpen Targets
0.09Suggestive
Alopecia universalisOpen Targets
0.09Suggestive
ringed hair diseaseOpen Targets
0.08Suggestive
melanomaOpen Targets
0.08Suggestive
hypotrichosis simplexOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
uncombable hair syndromeOpen Targets
0.07Suggestive
alopecia, androgenetic, 1Open Targets
0.07Suggestive
pili geminiOpen Targets
0.07Suggestive
Graham Little-Piccardi-Lassueur syndromeOpen Targets
0.07Suggestive
hypotrichosis 4Open Targets
0.07Suggestive
alopecia universalis congenitaOpen Targets
0.07Suggestive
wooly hair, autosomal recessive 3Open Targets
0.07Suggestive
hypotrichosis 1Open Targets
0.06Suggestive
monilethrix-1Open Targets
0.06Suggestive
hypotrichosis 15Open Targets
0.06Suggestive
Trichodysplasia - xerodermaOpen Targets
0.06Suggestive
trichodysplasia-xeroderma syndromeOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PALMProtein interaction83%C18orf32Shared pathway50%LURAP1LShared pathway50%TMEM101Shared pathway50%TMEM9BShared pathway50%CC2D1AShared pathway50%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
56%
Heart
50%
Ovary
38%
Lung
31%
Liver
12%
Gene Interaction Network
Click a node to explore
ZDHHC13PALMC18orf32LURAP1LTMEM101TMEM9BCC2D1A
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8IUH4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.31LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.09 [0.91–1.31]
RankingsWhere ZDHHC13 stands among ~20K protein-coding genes
  • #11,096of 20,598
    Most Researched35
  • #13,818of 17,882
    Most Constrained (LOEUF)1.31
Genes detectedZDHHC13
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Palmitic Acid Accelerates Endothelial Cell Injury and Cardiovascular Dysfunction via Palmitoylation of PKM2.
PMID: 39665133
Adv Sci (Weinh) · 2025
1.00
2
Palmitoylation-dependent activation of MC1R prevents melanomagenesis.
PMID: 28869973
Nature · 2017
0.90
3
The ZDHHC13/ZDHHC17 subfamily: From biological functions to therapeutic targets of diseases.
PMID: 39306022
Pharmacol Res · 2024
0.80
4
DNA Methylation-Regulated ZDHHC13 Promotes the Progression of Parkinson's Disease.
PMID: 40088380
Mol Neurobiol · 2025
0.70
5
Palmitoylation of ULK1 by ZDHHC13 plays a crucial role in autophagy.
PMID: 39169022
Nat Commun · 2024
0.60