Relevance of Abnormal KCNN1 Expression and Osmotic Hypersensitivity in Ewing Sarcoma.

ABSTRACT: Ewing sarcoma (EwS) is a rare and highly malignant bone tumor occurring mainly in childhood and adolescence. Physiologically, the bone is a central hub for Ca²⁺ homeostasis, which is severely disturbed by osteolytic processes in EwS. Therefore, we aimed to investigate how ion transport proteins involved in Ca²⁺ homeostasis affect EwS pathophysiology. We characterized the expression of 22 candidate genes of Ca²⁺-permeable or Ca²⁺-regulated ion channels in three EwS cell lines and found the Ca²⁺-activated K⁺ channel K_Ca2.1 (KCNN1) to be exceptionally highly expressed. We revealed that KCNN1 expression is directly regulated by the disease-driving oncoprotein EWSR1-FL1. Due to its consistent overexpressi

SUBMITTER: Fuest S 

PROVIDER: S-EPMC9564116 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature