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6 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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H4C4
H4 clustered histone 4
Chromosome 6 · 6p22.2
NCBI Gene: 8360Ensembl: ENSG00000158406.6HGNC: HGNC:20510UniProt: P62805
129PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Swiss-Prot Reviewed
infectious diseaseviral diseaseTessadori-Van Haaften neurodevelopmental syndrome 3breast carcinoma
✦AI Summary

H4C4 encodes histone H4, a core component of the nucleosome that organizes DNA and regulates chr6 structure essential for DNA replication, transcription, and repair 1. The H4 protein forms part of the histone octamer around which DNA wraps, with specific amino acid residues in the globular domain and C-terminal tail mediating protein-protein interactions with other histones and histone chaperones 1. De novo missense mutations in H4C4 and related H4 genes cause a neurodevelopmental syndrome characterized by intellectual disability, motor delay, and developmental delay 1. Functional studies in zebrafish revealed that pathogenic variants induce abnormal development, defective head organs, and reduced body axis length, establishing H4 variants as significant causes of developmental pathology 1. These findings position H4 missense mutations alongside histone H3 variants as emerging causes of chr6-related developmental disorders. H4C4 also serves as a marker (CD44) in developmental and pathological contexts. Altered H4C4/CD44 expression patterns characterize tethered spinal cord and myelomeningocele, suggesting its role in neural tube patterning 23. Additionally, CD44 mediates cell adhesion and hyaluronan binding, functions relevant to cancer biology where CD44 expression correlates with tumor progression and poor prognosis 456.

Sources cited
1
H4C4 encodes histone H4, a core nucleosome protein; de novo missense variants in H4C4 cause neurodevelopmental syndrome with intellectual disability and developmental delay; variants cluster in the H4 globular domain affecting histone-histone and histone-chaperone interactions
PMID: 35202563
2
H4C4/CD44 exhibits altered immunoexpression in tethered filum terminale compared to controls, indicating a role in neural development
PMID: 14512685
3
H4C4 serves as a caudal spinal cord developmental marker; altered H4C4 expression patterns are associated with abnormal patterning in myelomeningocele
PMID: 14512686
4
H4C4 monoclonal antibody recognizes CD44, an 85-kDa glycoprotein involved in homotypic cell aggregation and cell-to-cell adhesion through cytoskeletal mechanisms
PMID: 1689752
5
H4C4 antibody detects CD44 expression on mesothelioma cells; CD44 mediates functional association with hyaluronan
PMID: 9166476
6
CD44s (H4C4 antigen) is upregulated in pancreatic cancer and correlates with poor patient survival; anti-CD44s antibodies reduce tumor initiation and metastasis
PMID: 24397969
Disease Associationsⓘ20
infectious diseaseOpen Targets
0.50Moderate
viral diseaseOpen Targets
0.50Moderate
Tessadori-Van Haaften neurodevelopmental syndrome 3Open Targets
0.46Moderate
breast carcinomaOpen Targets
0.39Weak
cancerOpen Targets
0.38Weak
melanomaOpen Targets
0.37Weak
dengue diseaseOpen Targets
0.37Weak
posterior cortical atrophyOpen Targets
0.08Suggestive
sebaceous of Jadassohn nevusOpen Targets
0.06Suggestive
breast cancerOpen Targets
0.06Suggestive
actinic keratosisOpen Targets
0.06Suggestive
squamous cell carcinomaOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.04Suggestive
acute respiratory distress syndromeOpen Targets
0.03Suggestive
malignant hyperthermia, susceptibility to, 1Open Targets
0.03Suggestive
cerebral creatine deficiency syndromeOpen Targets
0.03Suggestive
lung cancerOpen Targets
0.02Suggestive
Ovarian EndometriosisOpen Targets
0.02Suggestive
ulcerative colitisOpen Targets
0.02Suggestive
systemic lupus erythematosusOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
H3C13Protein interaction100%H3C12Protein interaction100%CENPAProtein interaction100%H2BC12Protein interaction100%H2BC11Protein interaction99%H2AC21Protein interaction98%
Tissue Expression6 tissues
Ovary
0%
Brain
0%
Bone Marrow
0%
Heart
0%
Lung
0%
Liver
0%
Gene Interaction Network
Click a node to explore
H4C4H3C13H3C12CENPAH2BC12H2BC11H2AC21
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt P62805
View on AlphaFold ↗
RankingsWhere H4C4 stands among ~20K protein-coding genes
  • #3,624of 20,598
    Most Researched129 · top quartile
Genes detectedH4C4
Sources retrieved6 papers
Response time—
📄 Sources
6▼
1
Recurrent de novo missense variants across multiple histone H4 genes underlie a neurodevelopmental syndrome.
PMID: 35202563
Am J Hum Genet · 2022
1.00
2
The immunohistochemical profile of the tethered filum terminale.
PMID: 14512685
Pediatr Neurosurg · 2003
0.83
3
The immunohistochemical profile of the myelomeningocele placode: is the placode normal?
PMID: 14512686
Pediatr Neurosurg · 2003
0.67
4
Homotypic cell aggregation induced by anti-CD44(Pgp-1) monoclonal antibodies and related to CD44(Pgp-1) expression.
PMID: 1689752
J Immunol · 1990
0.50
5
High CD44 expression on human mesotheliomas mediates association with hyaluronan.
PMID: 9166476
Cancer J Sci Am · 1995
0.33