Table. List of Genes, Targeted Mutations of Which Lead to Artery Calcification in Mice With Respective Association Studies in Humans.

Gene Symbol, Human Protein Name, [OMIM Entry No.], Human Gene Map Locus	Mouse Model	Major Phenotypic Features	SNP-Based Association Studies in Humans	References
MGP, matrix Gla protein, [154870], 12p13.1-p12.3	mgp−/−	Arterial and cartilage calcification, tracheobronchial stenosis. Human correlate: Keutel syndrome	Increased CAC in men	73, 121, 122, 170
ENPP1, Ectonucleototide pyrophosphatase/ phosphodiesterase1, [173335], 6q22-q23	ttw/ttw enpp1−/−	Articular cartilage calcification, hyperostosis, spine and peripheral joint fusion, arterial calcification. Human correlate: Generalized arterial calcification of infancy	Increased aortic arch calcification in patients with type II diabetes Higher coronary calcification scores in ESRD patients	92, 93, 153, 154
ANKH, Progressive ankylosis [605145], 5p15.2-p14.1	ank/ank	Articular cartilage calcification, hyperostosis, spine and peripheral joint fusion, arterial calcification. Human correlate: Craniometaphysial dysplasia, Chondrocalcinosis-2		97
OPG, Osteoprotegerin, [602643], 8q24	opg−/−	Osteoporosis, vascular calcification	Increased risk for coronary artery disease	83-86
Smad6/Madh6, SMA- and MAD-related protein 6 [602931], 15q21-q22	madh6-mutant	Endocardial cushion defects, aortic ossification		38, 102
FBN1, Fibrillin1 [134797], 15q21.1	mgΔ/mgΔ, mgR/mgR	Aortic aneurysm, long bone overgrowth, artery media calcification. Human correlate: Marfan syndrome		103
CA2, Carbonic anhydrase2 [259730], 8q22	car-2−/−	Osteopetrosis, renal tubular acidosis, calcification of media of small arteries. Human correlate: Carbonic anhydrase II deficiency		99
KL, Klotho [604824], 13q12	klotho−/−	Vascular calcification, accelerated aging	Early onset of coronary artery disease	81, 82
AHSG, α2-HS-glycoprotein/fetuin, [138680], 3q27	ahsg−/−	Widespread but mild vascular calcification	Coronary artery calcified plaques in type II diabetes patients	79, 171
SPP1, Osteopontin, [166490], 4q21-q25	opn−/−	Enhanced calcification of cardiac valve implants		72, 113
ABCC6, ATP-binding cassette transporter subtype 6 [264800], 16p13.1	abcc6−/−	Myocardial necrosis and calcification, arterial calcification after freeze/thaw injury. Human correlate: Pseudoxanthoma elasticum		58, 60, 61
TIF1α, transcriptional intermediary factor 1 [603406], 7q32-q34	Tif1α−/−	Calcification of arterioles, arteries, vibrissae and lungs		37
ADIPOQ, Adiponectin [605441], 3q27	adiponectin−/−	Calcification of the aortic media in 30-week-old mice on chow diet		108, 110