cardiovascular system
N |
• slope compliance (reflecting noncontractile biomechanical properties) and blood pressure are not different from controls
|
• ATP-sensitive potassium [K(ATP)] channel density is reduced in aortic smooth muscle
|
• aortic diameter is greater at all pressures, similarly to that seen in heterozygotes
|
• K(ATP) channel density is dramatically lower in myocytes
|
• hearts are larger but not obviously more so than in heterozygotes
|
• chamber dilation
|
• lower basal K(ATP) conductance in isolated aortic smooth muscle cells
|
• K(ATP) current is reduced in ventricular myocytes to a greater extent than in heterozygotes
|
growth/size/body
• hearts are larger but not obviously more so than in heterozygotes
|
homeostasis/metabolism
• pinacidil has almost no effect on lowering blood pressure as it does in controls and heterozygous mice
• pinacidil-mediated activation is essentially absent in cardiomyocytes, while relative diazoxide-mediated activation is enhanced indicating that pharmacological sensitivity of myocardial fibers is switched
|
muscle
• ATP-sensitive potassium [K(ATP)] channel density is reduced in aortic smooth muscle
|
• K(ATP) channel density is dramatically lower in myocytes
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
hypertrichotic osteochondrodysplasia Cantu type | DOID:0060569 |
OMIM:239850 |
J:308986 |