OpenOnco
UA EN

Onco Wiki / Biomarker

IDH1 R132C mutation (glioma / cholangiocarcinoma / AML)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBIO-IDH1-R132C
TypeBiomarker
Aliases
IDH1 R132CМутація IDH1 R132C (гліома / холангіокарцинома / ГМЛ)
Statusreviewed 2026-04-29 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-EANO-GBM-2024 SRC-ESMO-AML-2020 SRC-NCCN-CNS-2025

Biomarker Facts

Biomarker typegene_mutation
Mutation details{"exon": "4", "functional_impact": "neomorphic — converts α-KG to 2-hydroxyglutarate (oncometabolite); DNA hypermethylation", "gene": "IDH1", "hgvs_protein": "p.R132C", "variant_type": "missense"}
Measurement
MethodNGS panel (R132C is IHC-negative with the R132H-specific antibody — requires sequencing). Liquid-biopsy ctDNA assays also detect R132C.
Unitscategorical (positive | negative)
Actionability lookup{"gene": "IDH1", "variant": "R132C"}
Related biomarkersBIO-IDH1-R132H BIO-IDH-MUTATION BIO-MGMT-METHYLATION

Notes

Second most common IDH1 variant. Distribution differs by disease: R132C is the dominant IDH1 variant in cholangiocarcinoma (~25-30% of IDH1-mutant CCA per ClarIDHy) and chondrosarcoma; in glioma it is rarer than R132H (<5%); in AML R132C accounts for ~30% of IDH1 mutations. Critically, R132C is IHC-NEGATIVE with the R132H-specific antibody (clone H09) — relying on IHC alone misses these patients. Ivosidenib (Tibsovo) is approved for IDH1-mutant cholangiocarcinoma (ClarIDHy 2L+) and IDH1-mutant AML (AGILE in combination with azacitidine 1L unfit; monotherapy R/R) — labels cover all IDH1 variants on the proven hotspot list (R132C/R132G/R132H/R132L/R132S), not just R132H. Reuses existing REG-IVOSIDENIB and IDH1-keyed indications via this biomarker entity.

Used By

Indications