Ontology highlight
ABSTRACT:
SUBMITTER: Szeri F
PROVIDER: S-EPMC7987643 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
Szeri Flora F Niaziorimi Fatemeh F Donnelly Sylvia S Orndorff Joseph J van de Wetering Koen K
FEBS letters 20201105 6
ABCC6 mediates release of ATP from hepatocytes into the blood. Extracellularly, ATP is converted into the mineralization inhibitor pyrophosphate. Consequently, inactivating mutations in ABCC6 give low plasma pyrophosphate and underlie the ectopic mineralization disorder pseudoxanthoma elasticum. How ABCC6 mediates cellular ATP release is still unknown. Fluorescent ABCC6 fusion proteins would allow mechanistic studies, but fluorophores attached to the ABCC6 N- or C-terminus result in intracellula ...[more]