LHX2

Molecular characteristics

Variants in LHX2 are inherited in an autosomal dominant manner, but to date most cases resulted from de novo variants. The affected individuals therefore usually present as sporadic cases, with a single occurrence in families.

Different types of variants have been reported as disease-causing in LHX2 (truncating variants and missense variants) and currently there is no correlation between the specific changes and the severity of the disease.