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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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RIPK4
receptor interacting serine/threonine kinase 4
Chromosome 21 Β· 21q22.3
NCBI Gene: 54101UniProt: Q96T11
72PubMed Papers
0Diseases
0Drugs
8Pathogenic Variants
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
intracellular signal transductionprotein bindingmorphogenesis of an epitheliumskin development
✦AI Summary

RIPK4 (receptor-interacting serine/threonine kinase 4) is a multifunctional kinase with distinct roles in development, homeostasis, and disease. Primarily, RIPK4 regulates epidermal differentiation through activation of LATS1/2 in the Hippo pathway via phase separation mechanisms, with inactivating mutations causing developmental syndromes with defective epidermal differentiation 1. RIPK4 also controls skeletal homeostasis by phosphorylating MFN2, promoting mitochondrial fragmentation that drives osteogenesis while restricting myelopoiesis through mitochondrial transfer regulation 2. Mechanistically, RIPK4 kinase activity is essential for ferroptotic cell death in response to oxidative stress, operating through downregulation of ACSM1 and modulation of polyunsaturated fatty acid metabolism 3. RIPK4 protein stability is regulated by GSK3Ξ²-mediated phosphorylation and UCHL3-dependent deubiquitination 4. Clinically, RIPK4 exhibits contradictory roles in cancer. In cutaneous squamous cell carcinoma, RIPK4 depletion paradoxically increases malignancy potential 5, while RIPK4 overexpression promotes ovarian cancer progression through epithelial-mesenchymal transition and invasion 6. BRAF kinase inhibitors reduce RIPK4 levels in melanoma cells 7. Notably, RIPK4 and RIPK1-3 remain understudied in neuroinflammation despite their importance in other RIPK family functions 8.

Sources cited
1
RIPK4 promotes epidermal differentiation through LATS1/2 activation via phase separation; inactivating mutations cause developmental syndromes with defective epidermal differentiation
PMID: 40570855
2
RIPK4 regulates skeletal homeostasis through MFN2-mediated mitochondrial dynamics, driving osteogenesis and restricting myelopoiesis; RIPK4 mutations cause Bartsocas-Papas syndrome
PMID: 40683865
3
RIPK4 kinase activity is required for oxidative stress-induced ferroptotic death via ACSM1 downregulation and PUFA metabolism; RIPK4 ablation protects from acute kidney injury
PMID: 39316049
4
GSK3Ξ² phosphorylates RIPK4 at Ser420 to enhance UCHL3-mediated deubiquitination and stabilization; RIPK4-UCHL3 axis promotes ovarian cancer metastasis
PMID: 38664501
5
RIPK4 depletion increases proliferation, migration, and invasion in cutaneous squamous cell carcinoma, indicating a tumor-suppressive role
PMID: 35186499
6
RIPK4 overexpression associates with advanced-stage ovarian cancer and poor prognosis; promotes invasion and epithelial-mesenchymal transition
PMID: 38179758
7
BRAF kinase inhibitors vemurafenib and dabrafenib downregulate RIPK4 protein levels in melanoma cells through off-target inhibition
PMID: 36765875
8
RIPK4, RIPK5, RIPK6, and RIPK7 have not been linked to neuroinflammation, unlike RIPK1, RIPK2, and RIPK3
PMID: 38349514
Pathogenic Variants8
NM_020639.3(RIPK4):c.722G>A (p.Arg241His)Likely pathogenic
Bartsocas-Papas syndrome 1
β˜…β˜†β˜†β˜†2021β†’ Residue 241
NM_020639.3(RIPK4):c.121del (p.His41fs)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2020β†’ Residue 41
NM_020639.3(RIPK4):c.1074dup (p.Glu359Ter)Pathogenic
Bartsocas-Papas syndrome 1
β˜†β˜†β˜†β˜†2021β†’ Residue 359
NM_020639.3(RIPK4):c.850G>A (p.Glu284Lys)Pathogenic
Curly hair, ankyloblepharon, nail dysplasia syndrome
β˜†β˜†β˜†β˜†2018β†’ Residue 284
NM_020639.3(RIPK4):c.242T>A (p.Ile81Asn)Pathogenic
Bartsocas-Papas syndrome 1
β˜†β˜†β˜†β˜†2012β†’ Residue 81
NM_020639.3(RIPK4):c.362T>A (p.Ile121Asn)Pathogenic
Bartsocas-Papas syndrome 1
β˜†β˜†β˜†β˜†2012β†’ Residue 121
NM_020639.3(RIPK4):c.777dup (p.Arg260fs)Pathogenic
Bartsocas-Papas syndrome 1
β˜†β˜†β˜†β˜†2012β†’ Residue 260
NM_020639.3(RIPK4):c.1127C>A (p.Ser376Ter)Pathogenic
Bartsocas-Papas syndrome 1
β˜†β˜†β˜†β˜†2012β†’ Residue 376
View on ClinVar β†—
Related Genes
PRKCDProtein interaction95%WDR90Protein interaction82%IRF6Protein interaction78%GREB1Shared pathway33%MYZAPShared pathway33%LRRC69Shared pathway33%
Tissue Expression

No tissue expression data available for this gene.

Gene Interaction Network
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RIPK4PRKCDWDR90IRF6GREB1MYZAPLRRC69
PROTEIN STRUCTURE
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AlphaFoldAI-predicted Β· UniProt Q96T11
View on AlphaFold β†—
RankingsWhere RIPK4 stands among ~20K protein-coding genes
  • #6,581of 20,598
    Most Researched72
  • #3,068of 5,498
    Most Pathogenic Variants8
Genes detectedRIPK4
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Ripks and Neuroinflammation.
PMID: 38349514
Mol Neurobiol Β· 2024
1.00
2
RIPK4 promotes oxidative stress and ferroptotic death through the downregulation of ACSM1.
PMID: 39316049
Proc Natl Acad Sci U S A Β· 2024
0.90
3
RIPK4 promotes epidermal differentiation through phase separation and activation of LATS1/2.
PMID: 40570855
Dev Cell Β· 2025
0.80
4
RIPK4-mediated MFN2 degradation drives osteogenesis through mitochondrial fragmentation and restricts myelopoiesis by blocking mitochondrial transfer.
PMID: 40683865
Nat Commun Β· 2025
0.70
5
GSK3Ξ² and UCHL3 govern RIPK4 homeostasis via deubiquitination to enhance tumor metastasis in ovarian cancer.
PMID: 38664501
Oncogene Β· 2024
0.60