Signaling pathways and inhibitors of cells from patients with kaposiform lymphangiomatosis.

ABSTRACT:

Background

Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly with significant morbidity and mortality. KLA is characterized by diffuse multifocal lesions comprised of focal areas of "kaposiform" spindled cells accompanying malformed lymphatic channels. The goal of this study was to identify activated signaling pathways in cells isolated from three KLA patients for the purpose of testing new therapies.

Procedure

Cells were obtained from the lungs of one patient isolated at autopsy and the spleen of two patients removed in surgery due to disease complications. A protein kinase array was performed on the KLA cell lysates and normal lymphatic endothelial cells.

Results

Higher activation of key signaling pathways in the KLA cells, including PRAS40, AKT1/2/3, a

SUBMITTER: Boscolo E 

PROVIDER: S-EPMC6588438 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature