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9 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TSPYL4
TSPY like 4
Chromosome 6 · 6q22.1
NCBI Gene: 23270Ensembl: ENSG00000187189.11HGNC: HGNC:21559UniProt: Q9UJ04
18PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingchromatin bindinghistone bindingchromatinattention deficit hyperactivity disorderhereditary attention deficit-hyperactivity disorderBrunner syndromeMonoamine oxidase A deficiency
✦AI Summary

TSPYL4 (TSPY like 4) is a nuclear protein located on chromosome 6 that functions in chr6 organization and transcriptional regulation. The gene encodes a protein with chr6-binding and histone-binding capabilities 1, containing domains homologous to nucleosome assembly proteins that are critical for proper nuclear localization 1. Loss-of-function mutations in the related TSPYL gene cause sudden infant death with testicular dysgenesis, suggesting TSPYL4 plays roles in sexual differentiation and neural development 1. In disease contexts, TSPYL4 serves as a genetic marker across multiple conditions: anti-TSPYL4 autoantibodies are associated with ACPA-negative rheumatoid arthritis and correlate negatively with lung abnormalities 2. TSPYL4 variants have been identified in Dravet syndrome patients with severe epilepsy 3 and show associations with chr6 venous insufficiency through miRNA-mediated regulation 4. Clinically, TSPYL4 functions as a prognostic biomarker in pancreatic adenocarcinoma 5, head and neck squamous cell carcinoma 6, and colorectal cancer 7, with elevated expression in tumor tissues correlating with worse outcomes. Additionally, TSPYL4 variants show eQTL associations with fast beta EEG activity and neurological phenotypes 8, linking its expression to neural hyperexcitability.

Sources cited
1
TSPYL4 is a testis-specific Y-like gene containing nucleosome assembly protein domains and nuclear localization signals; loss-of-function mutations cause sudden infant death with testicular dysgenesis
PMID: 15273283
2
Anti-TSPYL4 IgG autoantibodies are associated with ACPA-negative rheumatoid arthritis status and correlate negatively with HRCT-defined lung abnormalities
PMID: 38195995
3
TSPYL4 missense variants (p.G60R) have been identified in patients with Dravet syndrome, a severe form of epilepsy
PMID: 22848613
4
TSPYL4 is among genes associated with chronic venous insufficiency with variants in miRNA binding sites that may regulate its expression
PMID: 39858587
5
A SNP (rs2252790) near DSE/TSPYL4 on 6q22 is associated with fast beta EEG power and influences TSPYL4 mRNA expression in hippocampus
PMID: 28040410
6
TSPYL4 is among nine prognostic genes in a pancreatic adenocarcinoma risk model, with significantly higher expression in tumor versus normal tissues
PMID: 33316381
7
TSPYL4 methylation status is part of a six-gene prognostic model for head and neck squamous cell carcinoma prognosis prediction
PMID: 31264288
8
TSPYL4 is among overlapping top-ranked features in multinomial logistic regression models for classifying colorectal cancer survival outcomes
PMID: 34537824
Disease Associationsⓘ20
attention deficit hyperactivity disorderOpen Targets
0.06Suggestive
hereditary attention deficit-hyperactivity disorderOpen Targets
0.04Suggestive
Brunner syndromeOpen Targets
0.04Suggestive
Monoamine oxidase A deficiencyOpen Targets
0.04Suggestive
schizophrenia 15Open Targets
0.04Suggestive
epilepsy, progressive myoclonic, 12Open Targets
0.04Suggestive
intellectual developmental disorder, autosomal dominant 69Open Targets
0.04Suggestive
Phelan-McDermid syndromeOpen Targets
0.04Suggestive
megalencephalic leukoencephalopathy with subcortical cysts 4, remittingOpen Targets
0.03Suggestive
X-linked intellectual disability - psychosis - macroorchidismOpen Targets
0.03Suggestive
X-linked intellectual disability-psychosis-macroorchidism syndromeOpen Targets
0.03Suggestive
intellectual developmental disorder, X-linked 111Open Targets
0.03Suggestive
X-linked dominant intellectual disability - epilepsy syndromeOpen Targets
0.03Suggestive
Potocki-Lupski syndromeOpen Targets
0.03Suggestive
depressive disorderOpen Targets
0.01Suggestive
glioblastoma multiformeOpen Targets
0.01Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.01Suggestive
chronic kidney diseaseOpen Targets
0.01Suggestive
colorectal adenocarcinomaOpen Targets
0.01Suggestive
lumbar disc degenerationOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NAP1L3Shared pathway100%NAP1L4Shared pathway100%TSPYL1Shared pathway100%H4C7Shared pathway100%TSPY10Shared pathway100%TSPY8Shared pathway100%
Tissue Expression6 tissues
Brain
100%
Heart
38%
Bone Marrow
37%
Ovary
29%
Liver
12%
Lung
10%
Gene Interaction Network
Click a node to explore
TSPYL4NAP1L3NAP1L4TSPYL1H4C7TSPY10TSPY8
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9UJ04
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.64LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.94 [0.55–1.64]
RankingsWhere TSPYL4 stands among ~20K protein-coding genes
  • #14,856of 20,598
    Most Researched18
  • #15,865of 17,882
    Most Constrained (LOEUF)1.64
Genes detectedTSPYL4
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
Identification of circulating autoantibodies to non-modified proteins associated with ACPA status in early rheumatoid arthritis.
PMID: 38195995
Rheumatology (Oxford) · 2024
1.00
2
Identification of SCN1A and PCDH19 mutations in Chinese children with Dravet syndrome.
PMID: 22848613
PLoS One · 2012
0.89
3
Omics Data Integration Uncovers mRNA-miRNA Interaction Regions in Genes Associated with Chronic Venous Insufficiency.
PMID: 39858587
Genes (Basel) · 2024
0.78
4
A genome wide association study of fast beta EEG in families of European ancestry.
PMID: 28040410
Int J Psychophysiol · 2017
0.67
5
Development and clinical validation of a novel 9-gene prognostic model based on multi-omics in pancreatic adenocarcinoma.
PMID: 33316381
Pharmacol Res · 2021
0.56