HomeAboutRankingsData Sources
Β© 2026 GeneE
🧬
GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
ZDHHC9
zDHHC palmitoyltransferase 9
Chromosome X Β· Xq26.1
NCBI Gene: 51114Ensembl: ENSG00000188706.14HGNC: HGNC:18475UniProt: Q9Y397
55PubMed Papers
21Diseases
0Drugs
21Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingpalmitoyltransferase activityprotein-cysteine S-palmitoyltransferase activityRas palmitoyltransferase activitysyndromic X-linked intellectual disability Raymond typegenetic disorderIntellectual disabilityepilepsy
✦AI Summary

ZDHHC9 is an X-linked palmitoyltransferase that catalyzes S-palmitoylation of diverse cellular proteins, playing critical roles in viral infection, cellular metabolism, and immune regulation. Mechanistically, ZDHHC9 palmitoylates target proteins at specific cysteine residues, with activity enhanced by reactive oxygen species (ROS) and facilitated by protein-protein interactions 1. In SARS-CoV-2 infection, ZDHHC9 works sequentially with ZDHHC20 to palmitoylate spike protein in the endoplasmic reticulum, protecting it from degradation and enabling viral fusion [UniProt]. ZDHHC9 also palmitoylates metabolic regulators: GLUT1 (cysteine 207) to maintain plasma membrane localization and promote glucose uptake in glioblastoma 2, and LDHA (cysteine 163) to enhance lactate production in pancreatic cancer 3. In innate immunity, ZDHHC9 palmitoylates gasdermin D (GSDMD, cysteine 191/192) to facilitate pyroptosis and inflammatory responses 41, and cGAS (cysteines 404/405) to activate the STING pathway 5. ZDHHC9 also palmitoylates Bip/GRP78 in bladder cancer, inhibiting the unfolded protein response 6. Disease relevance includes X-linked intellectual developmental disorder (syndromic, Raymond type) and oncogenic roles in multiple cancers where ZDHHC9 upregulation correlates with poor prognosis 263. ZDHHC9 emerges as a potential therapeutic target for cancer and infectious diseases.

Sources cited
1
Mechanistically, ZDHHC9 palmitoylates target proteins at specific cysteine residues, with activity enhanced by reactive oxygen species (ROS) and facilitated by protein-protein interactions .
PMID: 38530158
2
ZDHHC9 also palmitoylates metabolic regulators: GLUT1 (cysteine 207) to maintain plasma membrane localization and promote glucose uptake in glioblastoma , and LDHA (cysteine 163) to enhance lactate production in pancreatic cancer .
PMID: 34620861
3
ZDHHC9 also palmitoylates metabolic regulators: GLUT1 (cysteine 207) to maintain plasma membrane localization and promote glucose uptake in glioblastoma , and LDHA (cysteine 163) to enhance lactate production in pancreatic cancer .
PMID: 38331089
4
In innate immunity, ZDHHC9 palmitoylates gasdermin D (GSDMD, cysteine 191/192) to facilitate pyroptosis and inflammatory responses , , and cGAS (cysteines 404/405) to activate the STING pathway .
PMID: 37802025
5
ZDHHC9 also palmitoylates Bip/GRP78 in bladder cancer, inhibiting the unfolded protein response .
PMID: 39002690
Disease Associationsβ“˜21
syndromic X-linked intellectual disability Raymond typeOpen Targets
0.78Strong
genetic disorderOpen Targets
0.50Moderate
Intellectual disabilityOpen Targets
0.46Moderate
epilepsyOpen Targets
0.37Weak
COVID-19Open Targets
0.37Weak
X-linked intellectual disability with marfanoid habitusOpen Targets
0.37Weak
neurodegenerative diseaseOpen Targets
0.23Weak
Neurodevelopmental disorderOpen Targets
0.12Weak
Neurodevelopmental abnormalityOpen Targets
0.11Weak
neoplasmOpen Targets
0.10Weak
breast cancerOpen Targets
0.09Suggestive
adenocarcinomaOpen Targets
0.08Suggestive
glioblastoma multiformeOpen Targets
0.08Suggestive
triple-negative breast cancerOpen Targets
0.07Suggestive
prostate cancerOpen Targets
0.07Suggestive
renal fibrosisOpen Targets
0.06Suggestive
cancerOpen Targets
0.06Suggestive
multiple myelomaOpen Targets
0.05Suggestive
papillary thyroid carcinomaOpen Targets
0.04Suggestive
GM2-gangliosidosis, AB variantOpen Targets
0.04Suggestive
Intellectual developmental disorder, X-linked, syndromic, Raymond typeUniProt
Pathogenic Variants21
NM_016032.4(ZDHHC9):c.442C>T (p.Arg148Trp)Pathogenic
Syndromic X-linked intellectual disability Raymond type|not provided|Intellectual disability
β˜…β˜…β˜†β˜†2024β†’ Residue 148
NM_016032.4(ZDHHC9):c.361C>T (p.Arg121Ter)Pathogenic
Syndromic X-linked intellectual disability Raymond type|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 121
NM_016032.4(ZDHHC9):c.267del (p.Ser89fs)Pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜…β˜†β˜†2023β†’ Residue 89
NM_016032.4(ZDHHC9):c.892C>T (p.Arg298Ter)Pathogenic
Inborn genetic diseases|Syndromic X-linked intellectual disability Raymond type
β˜…β˜…β˜†β˜†2023β†’ Residue 298
NM_016032.4(ZDHHC9):c.286C>T (p.Arg96Trp)Pathogenic
Syndromic X-linked intellectual disability Raymond type|not provided
β˜…β˜…β˜†β˜†2021β†’ Residue 96
NM_016032.4(ZDHHC9):c.811C>T (p.Gln271Ter)Likely pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜†β˜†β˜†2025β†’ Residue 271
NM_016032.4(ZDHHC9):c.777+1G>ALikely pathogenic
Syndromic X-linked intellectual disability Raymond type|Thyroid cancer, nonmedullary, 1
β˜…β˜†β˜†β˜†2024
NM_016032.4(ZDHHC9):c.488-2A>GLikely pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜†β˜†β˜†2024
NM_016032.4(ZDHHC9):c.792G>A (p.Trp264Ter)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2022β†’ Residue 264
NM_016032.4(ZDHHC9):c.881+1G>CLikely pathogenic
Intellectual disability
β˜…β˜†β˜†β˜†2020
NM_016032.4(ZDHHC9):c.717_738del (p.Val240fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2019β†’ Residue 240
NM_016032.4(ZDHHC9):c.743dup (p.Leu249fs)Pathogenic
Inborn genetic diseases
β˜…β˜†β˜†β˜†2018β†’ Residue 249
NM_016032.4(ZDHHC9):c.328+1G>APathogenic
not provided
β˜…β˜†β˜†β˜†2018
NM_016032.4(ZDHHC9):c.679del (p.Val226_Leu227insTer)Pathogenic
Inborn genetic diseases
β˜…β˜†β˜†β˜†2017β†’ Residue 226
NM_016032.4(ZDHHC9):c.852dup (p.Glu285Ter)Likely pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜†β˜†β˜†2016β†’ Residue 285
NM_016032.4(ZDHHC9):c.251T>C (p.Leu84Ser)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2015β†’ Residue 84
NM_016032.4(ZDHHC9):c.167+1G>ALikely pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜†β˜†β˜†
NM_016032.4(ZDHHC9):c.268G>A (p.Asp90Asn)Likely pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜…β˜†β˜†β˜†β†’ Residue 90
NM_016032.4(ZDHHC9):c.167+5G>CPathogenic
Syndromic X-linked intellectual disability Raymond type
β˜†β˜†β˜†β˜†2009
NM_016032.4(ZDHHC9):c.172_175del (p.Arg58fs)Pathogenic
Syndromic X-linked intellectual disability Raymond type
β˜†β˜†β˜†β˜†2009β†’ Residue 58
View on ClinVar β†—
Related Genes
ORMDL3Protein interaction100%SPTSSAProtein interaction100%SPTSSBProtein interaction100%SPTLC3Protein interaction94%NRASProtein interaction94%ZFP36L2Protein interaction91%
Tissue Expression6 tissues
Brain
100%
Liver
76%
Lung
47%
Ovary
22%
Heart
21%
Bone Marrow
17%
Gene Interaction Network
Click a node to explore
ZDHHC9ORMDL3SPTSSASPTSSBSPTLC3NRASZFP36L2
PROTEIN STRUCTURE
Preparing viewer…
PDB8HF3 Β· 3.40 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.29Highly Constrained
pLIβ“˜
1.00Intolerant
Observed/Expected LoF0.11 [0.05–0.29]
RankingsWhere ZDHHC9 stands among ~20K protein-coding genes
  • #8,272of 20,598
    Most Researched55
  • #2,154of 5,498
    Most Pathogenic Variants21
  • #1,074of 17,882
    Most Constrained (LOEUF)0.29 Β· top 10%
Genes detectedZDHHC9
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
ROS-dependent S-palmitoylation activates cleaved and intact gasdermin D.
PMID: 38599239
Nature Β· 2024
1.00
2
DHHC9-mediated GLUT1 S-palmitoylation promotes glioblastoma glycolysis and tumorigenesis.
PMID: 34620861
Nat Commun Β· 2021
0.90
3
The palmitoylation of gasdermin D directs its membrane translocation and pore formation during pyroptosis.
PMID: 38530158
Sci Immunol Β· 2024
0.80
4
ZDHHC9-mediated Bip/GRP78 S-palmitoylation inhibits unfolded protein response and promotes bladder cancer progression.
PMID: 39002690
Cancer Lett Β· 2024
0.70
5
RAB27B controls palmitoylation-dependent NRAS trafficking and signaling in myeloid leukemia.
PMID: 37317963
J Clin Invest Β· 2023
0.60