skeleton
• in 6-month-old mice, a 2-fold decrease in bone formation rate (BFR) is observed; similar results are seen in 2- and 4-month-old animals (88.6 um3/year vs 60.5 um3/year)
|
• heterozygotes have significantly decreased bone volume compared to wild-type
|
• less severe than in homozygotes
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• bone formation is abnormal, but bone resorption does not appear to be affected
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• delay is milder in heterozygotes
|
cellular
• in 6-month-old mice, a 2-fold decrease in bone formation rate (BFR) is observed; similar results are seen in 2- and 4-month-old animals (88.6 um3/year vs 60.5 um3/year)
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
osteoporosis-pseudoglioma syndrome | DOID:0060849 |
OMIM:259770 |
J:75973 |