Gene

H3-3A

H3.3 histone A

Explore in graph →CI-GENE-00004001HGNC:4764 ENSG00000163041 NCBI 3020 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (55)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 51–55 of 55 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
H3-3A K28M1
Dordaviprone	Glioma	Predictive	B	Supports Sensitivity Response	4	accepted	EID7601 In a phase 2 trial, patients with H3 K27M mutant glioma have been treated with DRD2 antagonist ONC201. Among 29 patients, 1 patient experienced complete clinical regression lasting >14 months, and 3 p…

Cohorts

Alteration frequency by cohort (26)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

gdcObserved26 cohort rows · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Thymoma TCGA-THYM	Thymoma	Ssm	24

Literature

Linked publications (8)

25 per page, newest first.

pubmedPublishedShowing 1–8 of 8 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

ONC201 (Dordaviprone) in Recurrent H3 K27M-Mutant Diffuse Midline Glioma.
· J Clin Oncol · 2024 · PMID 38335473 · validated civic_curation pubmed
Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone.
· Nat Genet · 2013 · PMID 24162739 · validated civic_curation pubmed
Pediatric low-grade gliomas can be molecularly stratified for risk.
· Acta Neuropathol · 2018 · PMID 29948154 · validated civic_curation pubmed
Diagnostic value of H3F3A mutations in giant cell tumour of bone compared to osteoclast-rich mimics.
· J Pathol Clin Res · 2015 · PMID 27499898 · validated civic_curation pubmed

H3-3A K28R2
(diagnostic)	Anaplastic AstrocytomaCURATED_BROADER	Diagnostic	C	Supports Positive	1	submitted	EID9836 Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 37 years of age anaplastic as… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC	civic
〃	OligodendrogliomaCURATED_BROADER	Diagnostic	C	Supports Positive	1	submitted	EID9838 Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 51 years of age oligodendrogl… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC	civic

H3-3A K28R2

(diagnostic)

Anaplastic AstrocytomaCURATED_BROADER

Diagnostic

Supports Positive

submitted

EID9836

Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 37 years of age anaplastic as… (full text at CIViC)

PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC

civic

〃

OligodendrogliomaCURATED_BROADER

Diagnostic

Supports Positive

submitted

EID9838

Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 51 years of age oligodendrogl… (full text at CIViC)

PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC

civic

H3-3A MUTATION2
(prognostic)	Childhood Low Grade Glioma	Prognostic	B	Supports Poor Outcome	4	accepted	EID7189 Using Sanger sequencing, H3F3A mutations in patients with pediatric low-grade gliomas (PLGGs) were identified (K27M: 13/280; G34R: 5/280). Kaplan-Meier survival analysis showed that H3F3A mutations we… (full text at CIViC) PMID 29948154 · Yang et al., 2018 · Open in CIViC	civic
(diagnostic)	Glioma	Diagnostic	B	Supports Positive	4	submitted	EID9417 Whole exome sequencing was performed on the glioblastomas of 48 patients (between 3 and 20 years of age). The authors also analyzed gliomas of 784 patients of various ages. The G34R/G34V variant has b… (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC	civic

H3-3A MUTATION2

(prognostic)

Childhood Low Grade Glioma

Prognostic

Supports Poor Outcome

accepted

EID7189

Using Sanger sequencing, H3F3A mutations in patients with pediatric low-grade gliomas (PLGGs) were identified (K27M: 13/280; G34R: 5/280). Kaplan-Meier survival analysis showed that H3F3A mutations we… (full text at CIViC)

PMID 29948154 · Yang et al., 2018 · Open in CIViC

civic

(diagnostic)

Glioma

Diagnostic

Supports Positive

submitted

EID9417

Whole exome sequencing was performed on the glioblastomas of 48 patients (between 3 and 20 years of age). The authors also analyzed gliomas of 784 patients of various ages. The G34R/G34V variant has b… (full text at CIViC)

PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC

civic