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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PWP2
PWP2 small subunit processome component
Chromosome 21 · 21q22.3
NCBI Gene: 5822Ensembl: ENSG00000241945.9HGNC: HGNC:9711UniProt: Q15269
98PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA bindingprotein bindingribosomal small subunit biogenesisnucleolusplacental retentionCrohn's diseaseprogressive myoclonus epilepsyhead and neck squamous cell carcinoma
✦AI Summary

PWP2 encodes a WD repeat-containing protein that functions as a component of the small subunit (SSU) processome, which is involved in ribosomal biogenesis 1. The protein localizes to the dense fibrillar component and granular component regions of nucleoli, where it participates in the assembly and maturation of the small ribosomal subunit 1. PWP2 shows low mobility in living cells and binds tightly to macro-protein complexes, suggesting it acts as a scaffold or core component within nucleolar complexes 1. The gene is located on chromosome 21.3 and consists of 21 exons spanning approximately 24-25 kb 23. PWP2 is ubiquitously expressed in all fetal and adult human tissues as a 3.3-3.4 kb transcript 43. The protein shows 42% identity to yeast PWP2 and belongs to the WD repeat-containing superfamily 3. Recent studies have identified PWP2 as a potential candidate gene for neurodevelopmental disorders, particularly those involving microcephaly, with variants potentially affecting cell cycle and division processes 5. The gene's location in the 21q22.3 region makes it a candidate for genetic disorders mapping to this chr21 region 2.

Sources cited
1
PWP2 is a WD repeat-containing protein component of SSU processome that localizes to nucleolar regions and acts as a scaffold in macro-protein complexes
PMID: 24754225
2
PWP2 gene consists of 21 exons spanning ~24 kb on chromosome 21q22.3 and is a candidate for genetic disorders in this region
PMID: 9205129
3
PWP2 shows 42% identity to yeast PWP2, belongs to WD repeat superfamily, and spans 25 kb with 21 exons
PMID: 8973917
4
PWP2 is ubiquitously expressed in all tissues as a 3.4 kb transcript
PMID: 8661145
5
PWP2 is identified as a potential candidate gene for neurodevelopmental disorders involving microcephaly and cell cycle processes
PMID: 37501076
Disease Associationsⓘ20
placental retentionOpen Targets
0.25Weak
Crohn's diseaseOpen Targets
0.13Weak
head and neck squamous cell carcinomaOpen Targets
0.01Suggestive
progressive myoclonus epilepsyOpen Targets
0.01Suggestive
genetic disorderOpen Targets
0.01Suggestive
Primary microcephalyOpen Targets
0.01Suggestive
cancerOpen Targets
0.00Suggestive
gastric adenocarcinomaOpen Targets
0.00Suggestive
melanomaOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
Cerebral cortical atrophyOpen Targets
0.00Suggestive
cerebral palsyOpen Targets
0.00Suggestive
cervical cancerOpen Targets
0.00Suggestive
Global developmental delayOpen Targets
0.00Suggestive
acute myeloid leukemiaOpen Targets
0.00Suggestive
Dravet syndromeOpen Targets
0.00Suggestive
Neurodevelopmental disorderOpen Targets
0.00Suggestive
trisomy 21Open Targets
0.00Suggestive
Alzheimer diseaseOpen Targets
0.00Suggestive
asthmaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MCCProtein interaction100%TTC27Protein interaction100%WDR18Protein interaction100%DHX37Protein interaction100%TFB2MProtein interaction100%GRWD1Protein interaction100%
Tissue Expression6 tissues
Lung
100%
Liver
81%
Ovary
80%
Brain
39%
Heart
17%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
PWP2MCCTTC27WDR18DHX37TFB2MGRWD1
PROTEIN STRUCTURE
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PDB7MQA · 2.70 Å · EM
View on RCSB ↗
RankingsWhere PWP2 stands among ~20K protein-coding genes
  • #4,905of 20,598
    Most Researched98 · top quartile
Genes detectedPWP2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Genomic organization and complete nucleotide sequence of the human PWP2 gene on chromosome 21.
PMID: 9205129
Genomics · 1997
1.00
2
Cloning the cDNA of human PWP2, which encodes a protein with WD repeats and maps to 21q22.3.
PMID: 8661145
Genomics · 1996
0.90
3
Isolation and genomic structure of a human homolog of the yeast periodic tryptophan protein 2 (PWP2) gene mapping to 21q22.3.
PMID: 8973917
Genome Res · 1996
0.80
4
Diagnostic yield and novel candidate genes for neurodevelopmental disorders by exome sequencing in an unselected cohort with microcephaly.
PMID: 37501076
BMC Genomics · 2023
0.70
5
Genomic organization and complete nucleotide sequence of the TMEM1 gene on human chromosome 21q22.3.
PMID: 9196060
Biochem Biophys Res Commun · 1997
0.60