LITERATURE · PEER REVIEWED

Atypical Biallelic Inheritance in "Dominant" Genes: Evidence From a Large-Scale Consanguineus Exome Cohort

American Journal of Medical Genetics Part A · 2026 · Peer reviewed

COMPLETE CITATION

Citation and identifiers

Taşdelen E, Tekbaş UC, Kolkıran A, Çetinkaya S, Kılıç M, Sezer A. “Atypical Biallelic Inheritance in "Dominant" Genes: Evidence From a Large-Scale Consanguineus Exome Cohort.” American Journal of Medical Genetics Part A. 200(9):2088–2098 · doi:10.1002/ajmg.a.70231.

PubMed ID
42316479

RESEARCH QUESTION

Can selected biallelic ASH1L variants produce an allelic or dosage context distinct from established heterozygous haploinsufficiency?

The human cohort places one ASH1L observation within a broader study of atypical biallelic findings, supporting allele-specific follow-up without establishing a general recessive ASH1L disorder.

INTERPRETATION BOUNDARY

Read the finding and limit together.

What this study supports

A retrospective consanguineous exome cohort included an ASH1L observation among selected biallelic findings in genes usually associated with dominant disease. The authors interpreted the ASH1L context as potentially hypomorphic and dosage-sensitive.

What it cannot establish

This selected cohort observation does not establish a broadly recessive ASH1L disorder, a biallelic severity rule, or a contradiction of the established autosomal-dominant haploinsufficiency relationship. The exact alleles and measured functional dosage still determine what can be concluded.

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