Table 2.
Mineralization phenotypes of PXE-related murine strains (based on [60]).
| Strain | Phenotype |
|---|---|
| 129S1/SvImJ | Fibroosseous bone lesions∗
Vibrissae mineralization |
|
| |
| C3H/HeJ | Epicardial fibrosis and mineralization Fibroosseous bone lesions∗ |
|
| |
| DBA/2J | Epicardial fibrosis and mineralization Fibroosseous bone lesions∗ Arterial mineralization |
|
| |
| KK/HIJ | Systemic mineralization (lung, retina) Epicardial fibrosis and mineralization Fibroosseous bone lesions∗ Arterial mineralization Vibrissae mineralization Hyperplasia (most common in pancreatic islets) [24009271] |
|
| |
| Abcc6−/− (targeted ablation) | Spontaneous calcification of EFs (mainly in arteries, the renal cortex, and the capsule surrounding the sinuses of the vibrissae) Normal plasma mineral levels Mineralization of dermal EFs and collagen Lower plasma HDL cholesterol Increased plasma creatinine |
|
| |
| Abcc6−/− (deletion of NBF1) | Spontaneous calcification of EFs (mainly in arteries, the renal cortex, and the capsule surrounding the sinuses of the vibrissae) Normal plasma mineral levels |
|
| |
| DCC or Dyscalc | Myocardial calcification Absence of vascular and vibrissa calcification |
∗This lesion was also found in strains without PXE-like mineralization and was not linked to EF calcification. Probably this lesion is thus not associated with PXE or Abcc6 was a strong modifier gene in KK/HIJ mice when mutated [60]. The other described lesions, apart from hyperplasia in the KK/HIJ mouse, can be linked to PXE. NBF1: nucleotide-binding fold 1.