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KAT6B – RASopathy

The histone acetyltransferase gene KAT6B was included in a targeted next-generation sequencing panel of 18 Ras-MAPK pathway genes for patients with RASopathy. In a cohort of 51 unrelated probands and four affected relatives, no pathogenic or likely pathogenic KAT6B variants were reported, and no familial segregation was observed (PMID:35418823). No experimental studies to date have linked KAT6B dysfunction to aberrant Ras-MAPK signaling or RASopathy phenotypes. Therefore, there is currently no evidence supporting a causal role for KAT6B in RASopathies, and further investigation is required to evaluate any potential association.

References

  • Molecular syndromology • 2022 • Comprehensive Genetic Analysis of RASopathy in the Era of Next-Generation Sequencing and Definition of a Novel Likely Pathogenic KRAS Variation. PMID:35418823

Evidence Based Scoring (AI generated)

Gene–Disease Association

Limited

No pathogenic KAT6B variants identified among 51 RASopathy probands and four affected relatives in a large NGS panel study ([PMID:35418823]).

Genetic Evidence

Limited

Targeted sequencing of KAT6B in 51 unrelated probands yielded no P/LP variants ([PMID:35418823]).

Functional Evidence

No evidence

No functional assays have demonstrated a role for KAT6B in Ras-MAPK signaling or RASopathy pathogenesis.