A matter of dosage: SF-1 in adrenocortical development and cancer

摘要

Steroidogenic factor-1 (SF-1), a nuclear receptor transcription factor, has a pivotal role in adrenal and gonadal development in humans and mice. It was previously shown that Sf-1 dosage critically regulates adrenal size and function in mice, while in humans mutation of a single SF-1 allele leads to adrenal and/or gonadal dysgenesis. Recent studies have elucidated the role of an increased SF-1 dosage as an important pathogenetic factor in childhood adrenocortical cancer and in experimental adrenocortical cancer in mice and identified genes that are regulated by SF-1 in a dosage-dependent fashion. Drugs targeting SF-1 transcriptional activity may then represent potential therapeutic tools to be associated to current chemotherapeutic regimens for the treatment of adrenocortical cancer in children.