Analysis Tools
mortality/aging
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• all mice die immediately after birth
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• non-Mendelian ratios or embryos are seen starting at E14.5, with fewer homozygotes than expected and multiple resorbing embryos seen at E18.5
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growth/size/body
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• smaller body size at E18.5
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• decrease in body weight at E18.5
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limbs/digits/tail
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• mice exhibit proximally displaced patellae at E18.5
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muscle
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• most tendons are seen in the forelimbs at E14.5 but at E16.5, forelimb has fewer tendons
• the extensor carpi radialis brevis, extensor carpi ulnaris, and extensor indicis proprius are absent in the E16.5 forelimb
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skeleton
| N |
• skeletal preparations show normal pattering with no differences in femur length at E18.5
• normal bone mineralization at E18.5, with normal mineralization of primary osteoblast cultures, normal femur bone mineral density and bone volume fraction of whole femurs, no differences in collagen content, mineral to collagen ratio, carbonate substitution, and mineral crystallinity
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• mice exhibit proximally displaced patellae at E18.5
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• most tendons are seen in the forelimbs at E14.5 but at E16.5, forelimb has fewer tendons
• the extensor carpi radialis brevis, extensor carpi ulnaris, and extensor indicis proprius are absent in the E16.5 forelimb
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• alteration of cervical spine curvature at E18.5
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• knees at E18.5 have reduced patella-trochlea interaction and thinner patellar tendons
• patellar tendons have regions of altered type I collagen fiber organization
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• forelimb and hindlimb contractures are fully penetrant in all mice at E18.5
• transecting the tendons at the palmar side of the wrist reduces the contracture, suggesting that it is due to imbalanced tension between flexor and extensor tendons due to the absence of extensor tendons
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Bruck syndrome | DOID:0060231 |
OMIM:259450 OMIM:609220 |
J:358139 | |
