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GeneE
27 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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PUF60
poly(U) binding splicing factor 60
Chromosome 8 Β· 8q24.3
NCBI Gene: 22827Ensembl: ENSG00000179950.15HGNC: HGNC:17042UniProt: E9PL19
220PubMed Papers
21Diseases
0Drugs
105Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTranscription Factor
RESEARCH IMPACT
TrendingVariant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
RNA bindingnucleoplasmprotein bindingidentical protein binding8q24.3 microdeletion syndromeintellectual disability-cardiac anomalies-short stature-joint laxity syndromeneurodegenerative diseasegenetic disorder
✦AI Summary

PUF60 is a multifunctional RNA-binding protein that plays critical roles in pre-mRNA splicing, cell cycle regulation, and cancer progression. The protein promotes alternative splicing by binding to pyrimidine tracts and 3'-splice sites of pre-mRNAs, particularly those involved in cell cycle control 1. PUF60 regulates the splicing of key cell cycle genes including CDC25C, where its knockdown leads to exon skipping and subsequent nonsense-mediated decay, ultimately inhibiting G2/M transition and cell proliferation 1. In cancer contexts, PUF60 functions as an oncogenic factor - it is overexpressed in lung adenocarcinoma and correlates with poor prognosis 1. The protein drives cancer progression by ensuring proper exon inclusion in proliferation-associated transcripts, and disruption of its RNA-binding activity induces apoptosis in cancer cells 2. Beyond cancer, PUF60 haploinsufficiency causes Verheij syndrome, a neurodevelopmental disorder characterized by growth retardation, intellectual disability, cardiac anomalies, and ocular coloboma 34. The syndrome results from de novo pathogenic variants in PUF60, though inherited cases have been reported 3. PUF60 has also been identified as a novel gene associated with developmental and epileptic encephalopathy with spike-wave activation in sleep 5.

Sources cited
1
PUF60 promotes cell cycle progression by regulating alternative splicing of CDC25C and is overexpressed in lung cancer with poor prognosis correlation
PMID: 37682709
2
PUF60 drives cancer progression by binding to 3' splice sites and promoting exon inclusion in proliferation-associated transcripts
PMID: 41411621
3
PUF60 haploinsufficiency causes Verheij syndrome with multiple congenital anomalies including cardiac, ocular, and skeletal abnormalities
PMID: 37303278
4
Verheij syndrome results from de novo PUF60 mutations and presents with growth retardation, intellectual disability, and cardiac defects
PMID: 30078240
5
PUF60 is identified as a novel gene associated with developmental and epileptic encephalopathy with spike-wave activation in sleep
PMID: 39096015
Disease Associationsβ“˜21
8q24.3 microdeletion syndromeOpen Targets
0.81Strong
intellectual disability-cardiac anomalies-short stature-joint laxity syndromeOpen Targets
0.58Moderate
neurodegenerative diseaseOpen Targets
0.53Moderate
genetic disorderOpen Targets
0.52Moderate
Intellectual disabilityOpen Targets
0.50Moderate
syndromic intellectual disabilityOpen Targets
0.46Moderate
dengue diseaseOpen Targets
0.37Weak
neurodevelopmental disorder with hypotonia and variable intellectual and behavioral abnormalitiesOpen Targets
0.27Weak
CHARGE syndromeOpen Targets
0.26Weak
Neurodevelopmental abnormalityOpen Targets
0.26Weak
breast cancerOpen Targets
0.08Suggestive
age-related macular degenerationOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
urinary bladder carcinomaOpen Targets
0.08Suggestive
renal cell carcinomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
X-linked retinal dysplasiaOpen Targets
0.07Suggestive
choroidal dystrophy, central areolar, 1Open Targets
0.06Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.06Suggestive
Stargardt diseaseOpen Targets
0.06Suggestive
Verheij syndromeUniProt
Pathogenic Variants105
NM_078480.3(PUF60):c.475G>A (p.Asp159Asn)Pathogenic
not provided|8q24.3 microdeletion syndrome
β˜…β˜…β˜†β˜†2026β†’ Residue 159
NM_078480.3(PUF60):c.541G>A (p.Glu181Lys)Pathogenic
not provided|8q24.3 microdeletion syndrome|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025β†’ Residue 181
NM_078480.3(PUF60):c.590dup (p.Arg198fs)Pathogenic
not provided|8q24.3 microdeletion syndrome|Inborn genetic diseases
β˜…β˜…β˜†β˜†2025β†’ Residue 198
NM_078480.3(PUF60):c.382_383del (p.Met128fs)Pathogenic
not provided|8q24.3 microdeletion syndrome
β˜…β˜…β˜†β˜†2025β†’ Residue 128
NM_078480.3(PUF60):c.612_630del (p.Asn207fs)Pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 207
NM_078480.3(PUF60):c.891dup (p.Arg298fs)Pathogenic
not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 298
NM_078480.3(PUF60):c.407_410del (p.Ile136fs)Pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 136
NM_078480.3(PUF60):c.619_637del (p.Asn207fs)Pathogenic
not provided|8q24.3 microdeletion syndrome
β˜…β˜…β˜†β˜†2025β†’ Residue 207
NM_078480.3(PUF60):c.271C>T (p.Gln91Ter)Pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 91
NM_078480.3(PUF60):c.449_457del (p.Ala150_Phe152del)Pathogenic
8q24.3 microdeletion syndrome|Inborn genetic diseases|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 150
NM_078480.3(PUF60):c.1342C>T (p.Arg448Ter)Pathogenic
8q24.3 microdeletion syndrome|See cases|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 448
NM_078480.3(PUF60):c.896T>A (p.Val299Glu)Likely pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 299
NM_078480.3(PUF60):c.24+1G>APathogenic
not provided
β˜…β˜…β˜†β˜†2024
NM_078480.3(PUF60):c.367C>T (p.Arg123Trp)Likely pathogenic
not provided|8q24.3 microdeletion syndrome
β˜…β˜…β˜†β˜†2023β†’ Residue 123
NM_078480.3(PUF60):c.628C>T (p.Gln210Ter)Pathogenic
8q24.3 microdeletion syndrome|Intellectual disability
β˜…β˜…β˜†β˜†2023β†’ Residue 210
NM_078480.3(PUF60):c.1471G>A (p.Gly491Arg)Pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2022β†’ Residue 491
NM_078480.3(PUF60):c.389G>A (p.Arg130His)Pathogenic
8q24.3 microdeletion syndrome|CHARGE syndrome|not provided
β˜…β˜…β˜†β˜†2021β†’ Residue 130
NM_078480.3(PUF60):c.713C>G (p.Ser238Ter)Pathogenic
not provided|8q24.3 microdeletion syndrome
β˜…β˜…β˜†β˜†2021β†’ Residue 238
NM_078480.3(PUF60):c.1381-2A>GLikely pathogenic
8q24.3 microdeletion syndrome|not provided
β˜…β˜…β˜†β˜†2017
NM_078480.3(PUF60):c.1105C>T (p.Gln369Ter)Likely pathogenic
8q24.3 microdeletion syndrome
β˜…β˜†β˜†β˜†2025β†’ Residue 369
View on ClinVar β†—
Related Genes
SF3B3Protein interaction100%SF3B2Protein interaction100%SF3A2Protein interaction100%SNRPA1Protein interaction100%SF3A3Protein interaction100%SF3B1Protein interaction100%
Tissue Expression6 tissues
Liver
100%
Brain
81%
Bone Marrow
73%
Heart
65%
Lung
63%
Ovary
48%
Gene Interaction Network
Click a node to explore
PUF60SF3B3SF3B2SF3A2SNRPA1SF3A3SF3B1
PROTEIN STRUCTURE
Preparing viewer…
PDB3UE2 Β· 1.23 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.29Highly Constrained
pLIβ“˜
1.00Intolerant
Observed/Expected LoF0.17 [0.11–0.29]
RankingsWhere PUF60 stands among ~20K protein-coding genes
  • #1,870of 20,598
    Most Researched220 Β· top 10%
  • #738of 5,498
    Most Pathogenic Variants105 Β· top quartile
  • #1,097of 17,882
    Most Constrained (LOEUF)0.29 Β· top 10%
Genes detectedPUF60
Sources retrieved27 papers
Response timeβ€”
πŸ“„ Sources
27β–Ό
1
PUF60 promotes cell cycle and lung cancer progression by regulating alternative splicing of CDC25C.
PMID: 37682709
Cell Rep Β· 2023
1.00
2
Solving the Etiology of Developmental and Epileptic Encephalopathy with Spike-Wave Activation in Sleep (D/EE-SWAS).
PMID: 39096015
Ann Neurol Β· 2024
0.90
3
PUF60-Regulated Isoform Switching of MAZ Modulates Gastric Cancer Cell Migration.
PMID: 40411278
Cancer Med Β· 2025
0.80
4
Novel PUF60 variant suggesting an interaction between Verheij and Cornelia de Lange syndrome: phenotype description and review of the literature.
PMID: 38273166
Eur J Hum Genet Β· 2024
0.80
5
PUF60-related developmental disorder: A case series and phenotypic analysis of 10 additional patients with monoallelic PUF60 variants.
PMID: 37303278
Am J Med Genet A Β· 2023
0.70