Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
SucoGt(KST050)Byg mutation
(0 available);
any
Suco mutation
(49 available)
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mortality/aging
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• Background Sensitivity: all die neonatally on a mixed 129P2/OlaHsd and C57BL/6 background compared to about 50% lethality on a mixed 129P2/OlaHsd, C57BL/6 and CD-1 background
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respiratory system
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
SucoGt(KST050)Byg mutation
(0 available);
any
Suco mutation
(49 available)
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SucoGt(KST050)Byg/SucoGt(KST050)Byg mice exhibit growth retardation and low bone mass with multiple fractures
mortality/aging
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• Background Sensitivity: about 50% die neonatally on a mixed 129P2/OlaHsd, C57BL/6, and CD-1 background compared to 100% lethality on a mixed 129P2/OlaHsd and C57BL/6 background
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• over 80% mortality by P10 with no mice surviving to weaning
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respiratory system
skeleton
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• flatter compared to controls
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• markedly thinner at P10
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• at P5 long bones are misshapen
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• at P10 cortical bone resembles immature woven bone with sparse and randomly oriented type I collagen fibrils
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• modest decrease in the amount of cortical bone in neonates
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• dramatically thinner cortices in long bones at P10
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• small immature osteoblasts are present in fracture calluses
• at P10 in tibiae osteoblasts lack extensive, well-organized rough endoplasmic reticulum (rER) cisternae
• osteoblasts have a fibroblastic appearance with a scarce, discontinuous rER network and a higher nuclear/cytoplasmic ratio
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• expression analysis indicates an increase in osteocyte numbers
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• decrease in the amount of trabecular bone in neonates and at P10
• decrease in the number of trabeculae and increase in trabecular separation at P10
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• inflexible joints at P5
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• wider sutures in the cranial vault
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• modestly delayed in neonates
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• in vivo and in culture osteoblasts display impaired differentiation
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• hyperplastic calluses consist largely of calcified cartilage with small, immature osteoblasts present throughout the callus and fracture site but mature osteoblasts and newly formed bone are absent
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• labeling with calcein, a marker of newly formed bone, results in punctate discontinuous labeling rather than the regular labeling seen in wild-type controls
• however, bone that does form is properly mineralized
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• primary calvarial osteoblasts show reduced alkaline phosphatase activity, sparsely formed collagen matrix, and significantly fewer bone nodules
• in culture primary calvarial osteoblasts grow faster but display impaired differentiation potential compared to wild-type osteoblasts
• decrease in the synthesis of type I collagen chains
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• modest increase in osteoblast apoptosis
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• expression analysis indicates decreased osteoclast activity
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• spontaneous fractures are seen at P5
• hyperplastic calluses suggesting ongoing fracture repair are seen throughout the axial and appendicular skeleton
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growth/size/body
behavior/neurological
cardiovascular system
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• around the forelimbs and cervical vertebrae at P5
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• around the cervical vertebrae at P5
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homeostasis/metabolism
renal/urinary system
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• modest glomerular hypercellularity
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immune system
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• expression analysis indicates decreased osteoclast activity
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hematopoietic system
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• expression analysis indicates decreased osteoclast activity
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craniofacial
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• wider sutures in the cranial vault
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• flatter compared to controls
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• markedly thinner at P10
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nervous system
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• around the cervical vertebrae at P5
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cellular
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• in vivo and in culture osteoblasts display impaired differentiation
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• primary calvarial osteoblasts show reduced alkaline phosphatase activity, sparsely formed collagen matrix, and significantly fewer bone nodules
• in culture primary calvarial osteoblasts grow faster but display impaired differentiation potential compared to wild-type osteoblasts
• decrease in the synthesis of type I collagen chains
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• modest increase in osteoblast apoptosis
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