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. 2019 Apr 30;20(9):2142. doi: 10.3390/ijms20092142

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© 2019 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Schematic representation of vitamin K metabolism and related compounds in hepatocytes. Arrows indicate differential expression of molecular targets as observed in ectopic mineralization pathophysiology. Note that dp-ucMGP (an extrahepatic VKDP) is mainly synthesized in VSMCs and chondrocytes, and is thereafter transported to the liver. Post-translational modification then takes place in hepatocytes as shown above. ABCC6: ATP-binding cassette transporter subfamily C member 6. ALP: alkaline phosphatase. AMP: adenosine monophosphate. ANKH: progressive ankylosis homolog protein. ATP: adenosine triphosphate. ENPP1: ectonucleotide pyrophosphatase-phosphodiesterase 1. GACI: generalized arterial calcification of infancy. GGCX: gamma-glutamyl carboxylase. GRP: gla-rich protein. MGP: matrix gla protein. OC: osteocalcin. Pi: inorganic phosphate. PPi: inorganic pyrophosphate. PXE: pseudoxanthoma elasticum. VKCFD1/2: vitamin K-dependent coagulation factor deficiency 1/2. (d)(p)(u)cVKDP: (de)(phosphorylated)(un)carboxylated vitamin K-dependent protein. VKORC1: vitamin K 2,3-epoxide reductase complex subunit 1. VSMC: vascular smooth muscle cell.