Article
Matrix Metalloproteinases Contribute to the Calcification Phenotype in Pseudoxanthoma Elasticum.
Biomolecules - 12 Apr 2023
Plümers Ricarda, Lindenkamp Christopher, Osterhage Michel Robin, Knabbe Cornelius, Hendig Doris
Abstract excerpt
Ectopic calcification and dysregulated extracellular matrix remodeling are prominent hallmarks of the complex heterogenous pathobiochemistry of pseudoxanthoma elasticum (PXE). The disease arises from mutations in ABCC6, an ATP-binding cassette transporter expressed predominantly in the liver. Neither its substrate nor the mechanisms by which it contributes to PXE are completely understood. The fibroblasts...
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