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Phenotypes Associated with This Genotype
Genotype
MGI:2183282
Allelic
Composition
Mgptm1Kry/Mgptm1Kry
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mgptm1Kry mutation (1 available); any Mgp mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• homozygotes develop to term but die by 2 months of age from rupture and subsequent hemorrhage of the thoracic and abdominal aorta (J:38867)
• homozygotes develop to term but die by 2 months of age from rupture and subsequent hemorrhage of the thoracic and abdominal aorta (J:38867)

cardiovascular system
• at 2 and 3 weeks of age, homozygotes show extensive calcification of the elastic lamellae in the media of the aortic wall as well as disrupted tissue architecture (J:38867)
• affected aortae exhibit cartilaginous metaplasia, typified by the presence of chondrocytes and metachromatic cartilage matrix (J:38867)
• in the media, chondrocytes are surrounded by hyaline cartilage and type II collagen fibrils, proteoglycans and matrix vesicles (J:38867)
• at 2 and 3 weeks of age, homozygotes show extensive calcification of the elastic lamellae in the media of the aortic wall as well as disrupted tissue architecture (J:38867)
• affected aortae exhibit cartilaginous metaplasia, typified by the presence of chondrocytes and metachromatic cartilage matrix (J:38867)
• in the media, chondrocytes are surrounded by hyaline cartilage and type II collagen fibrils, proteoglycans and matrix vesicles (J:38867)
• affected aortae exhibit extensive calcification of elastic fibers and collagen fibrils in the media of the aortic wall (J:38867)
• the inorganic material found in elastic lamellae is identified as apatite (J:38867)
• affected aortae exhibit extensive calcification of elastic fibers and collagen fibrils in the media of the aortic wall (J:38867)
• the inorganic material found in elastic lamellae is identified as apatite (J:38867)
• in affected aortae, smooth muscle cells fail to retain their spatial arrangement as discrete, contiguous layers throughout the vessel wall (J:38867)
• in affected aortae, smooth muscle cells fail to retain their spatial arrangement as discrete, contiguous layers throughout the vessel wall (J:38867)
• at 2 and 3 weeks of age, homozygotes show widespread calcification along the aorta and its branches; no calcification is detected up to 1 week of age (J:38867)
• calcification originates at several sites and involves all elastic and muscular arteries, but not arterioles, capillaries or veins (J:38867)
• notably, calcification occurs in the absence of fatty streak or atherosclerotic plaques (J:38867)
• at 2 and 3 weeks of age, homozygotes show widespread calcification along the aorta and its branches; no calcification is detected up to 1 week of age (J:38867)
• calcification originates at several sites and involves all elastic and muscular arteries, but not arterioles, capillaries or veins (J:38867)
• notably, calcification occurs in the absence of fatty streak or atherosclerotic plaques (J:38867)
• kidneys are more vascularized than controls (J:176031)
• renal vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• kidney vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the kidneys (J:176031)
• kidneys are more vascularized than controls (J:176031)
• renal vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• kidney vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the kidneys (J:176031)
• total capillary density and the number of capillaries with a diameter of more than 20 um is increased in glomeruli (J:176031)
• total capillary density and the number of capillaries with a diameter of more than 20 um is increased in glomeruli (J:176031)
• pulmonary vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• lung vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the lungs (J:176031)
• pulmonary vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• lung vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the lungs (J:176031)
• mutants exhibit pulmonary and renal arteriovenous malformations (J:176031)
• presence of renal and pulmonary arteriovenous shunts (J:176031)
• mutants exhibit abnormal arteriovenous connections in the glomeruli, direct connections between an arterial segment and a venous segment are seen on the surface of the lungs and both afferent and efferent arterioles are seen in the kidneys (J:176031)
• mutants exhibit pulmonary and renal arteriovenous malformations (J:176031)
• presence of renal and pulmonary arteriovenous shunts (J:176031)
• mutants exhibit abnormal arteriovenous connections in the glomeruli, direct connections between an arterial segment and a venous segment are seen on the surface of the lungs and both afferent and efferent arterioles are seen in the kidneys (J:176031)
• mutants exhibit pulmonary arteriovenous malformations and shunts (J:176031)
• mutants exhibit pulmonary arteriovenous malformations and shunts (J:176031)
• at 2 and 3 weeks of age, homozygotes exhibit calcification in coronary arteries and aortic valves but not in the myocardium (J:38867)
• at 2 and 3 weeks of age, homozygotes exhibit calcification in coronary arteries and aortic valves but not in the myocardium (J:38867)
• homozygotes die from rupture and subsequent hemorrhage of the thoracic and abdominal aorta (J:38867)
• homozygotes die from rupture and subsequent hemorrhage of the thoracic and abdominal aorta (J:38867)
• beginning at 2 weeks of age, homozygotes display a significantly faster heart rate than wild-type mice (J:38867)
• beginning at 2 weeks of age, homozygotes display a significantly faster heart rate than wild-type mice (J:38867)

growth/size/body
• starting at 2 weeks of age, homozygotes exhibit a shorter stature relative to wild-type mice (J:38867)
• at death, homozygotes are noticeably shorter than wild-type mice (J:38867)
• starting at 2 weeks of age, homozygotes exhibit a shorter stature relative to wild-type mice (J:38867)
• at death, homozygotes are noticeably shorter than wild-type mice (J:38867)
• homozygotes exhibit a normal body size up to 2-3 weeks of age, but grow slowly thereafter (J:38867)
• homozygotes exhibit a normal body size up to 2-3 weeks of age, but grow slowly thereafter (J:38867)

skeleton
• homozygotes display inappropriate calcification of cartilage in the lower end of the trachea (J:38867)
• homozygotes display inappropriate calcification of cartilage in the lower end of the trachea (J:38867)
• at 8 weeks, homozygotes display inappropriate calcification of the growth-plate cartilage (J:38867)
• at 8 weeks, homozygotes display inappropriate calcification of the growth-plate cartilage (J:38867)
• at 8 weeks, calcification of the growth plate abnormally extends into the zone of proliferating chondrocytes (J:38867)
• mutant proliferating chondrocytes are not organized in columns as in wild-type growth plate (J:38867)
• at 8 weeks, calcification of the growth plate abnormally extends into the zone of proliferating chondrocytes (J:38867)
• mutant proliferating chondrocytes are not organized in columns as in wild-type growth plate (J:38867)
• abnormal calcification of the growth plate cartilage results in the absence of hypertrophic chondorcytes (J:38867)
• abnormal calcification of the growth plate cartilage results in the absence of hypertrophic chondorcytes (J:38867)
• abnormal calcification of the growth-plate cartilage leads to osteopenia and fractures (J:38867)
• abnormal calcification of the growth-plate cartilage leads to osteopenia and fractures (J:38867)

respiratory system
• mutants exhibit a paucity of terminal airways (J:176031)
• mutants exhibit a paucity of terminal airways (J:176031)
• pulmonary vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• lung vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the lungs (J:176031)
• pulmonary vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• lung vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the lungs (J:176031)
• lungs are slightly smaller (J:176031)
• lungs are slightly smaller (J:176031)
• homozygotes display inappropriate calcification of cartilage in the main bronchi (J:38867)
• homozygotes display inappropriate calcification of cartilage in the main bronchi (J:38867)
• homozygotes display inappropriate calcification of cartilage in the lower end of the trachea (J:38867)
• homozygotes display inappropriate calcification of cartilage in the lower end of the trachea (J:38867)

muscle
• in affected aortae, smooth muscle cells fail to retain their spatial arrangement as discrete, contiguous layers throughout the vessel wall (J:38867)
• in affected aortae, smooth muscle cells fail to retain their spatial arrangement as discrete, contiguous layers throughout the vessel wall (J:38867)

renal/urinary system
• kidneys are more vascularized than controls (J:176031)
• renal vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• kidney vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the kidneys (J:176031)
• kidneys are more vascularized than controls (J:176031)
• renal vasculature exhibits excessive branching of small vessels, irregular caliber of arteries, and evidence of arteriovenous malformations (J:176031)
• kidney vessels are enlarged (J:176031)
• some mutants exhibit enlarged niduses and entangled vessels in the kidneys (J:176031)
• kidneys show more glomeruli per microscopic field than controls (J:176031)
• mutants exhibit abnormal arteriovenous connections in the glomeruli (J:176031)
• kidneys show more glomeruli per microscopic field than controls (J:176031)
• mutants exhibit abnormal arteriovenous connections in the glomeruli (J:176031)
• total capillary density and the number of capillaries with a diameter of more than 20 um is increased in glomeruli (J:176031)
• total capillary density and the number of capillaries with a diameter of more than 20 um is increased in glomeruli (J:176031)
• kidneys are heavier when normalized to total body weight (J:176031)
• kidneys are heavier when normalized to total body weight (J:176031)
• kidneys are slightly smaller (J:176031)
• kidneys are slightly smaller (J:176031)


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory