H4C5

Chr 6AD

H4 clustered histone 5

Also known as: H4/j, H4FJ, HIST1H4E, TEBIVANED3, TEVANED3

Histones are basic nuclear proteins that are responsible for the nucleosome structure of the chromosomal fiber in eukaryotes. Two molecules of each of the four core histones (H2A, H2B, H3, and H4) form an octamer, around which approximately 146 bp of DNA is wrapped in repeating units, called nucleosomes. The linker histone, H1, interacts with linker DNA between nucleosomes and functions in the compaction of chromatin into higher order structures. This gene is intronless and encodes a replication-dependent histone that is a member of the histone H4 family. Transcripts from this gene lack polyA tails but instead contain a palindromic termination element. This gene is found in the large histone gene cluster on chromosome 6. [provided by RefSeq, Aug 2015]

Primary Disease Associations & Inheritance

Tessadori-Bicknell-van Haaften neurodevelopmental syndrome 3MIM #619950
AD
62
ClinVar variants
13
Pathogenic / LP
pLI score
0
Active trials
Clinical SummaryH4C5
🧬
Gene-Disease Validity (ClinGen)
Tessadori-Van Haaften neurodevelopmental syndrome 3 · ADStrong

Strong evidence — appropriate for clinical testing

📋
ClinVar Variants
13 Pathogenic / Likely Pathogenic· 42 VUS of 62 total submissions
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

62 submitted variants in ClinVar

Classification Summary

Pathogenic8
Likely Pathogenic5
VUS42
Likely Benign5
Benign1
Conflicting1
8
Pathogenic
5
Likely Pathogenic
42
VUS
5
Likely Benign
1
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
4
4
0
8
Likely Pathogenic
0
5
0
0
5
VUS
3
26
13
0
42
Likely Benign
1
0
1
3
5
Benign
0
0
0
1
1
Conflicting
1
Total43518462

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

H4C5 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

Tessadori-Bicknell-van Haaften neurodevelopmental syndrome 3

MIM #619950

Molecular basis of disorder known

Autosomal dominant
Clinical Literature
Landmark / reviewRecent case evidence
Key Publications
Landmark & review papers · by relevance
PubMed
Top 10 resultsSearch PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →