Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm1Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
|
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Lmnatm1Lgf/Lmnatm1Lgf mice are small and have poorly mineralized bone
mortality/aging
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• all mice die by 3 to 4 weeks of age
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skeleton
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• mice develop fractures in the extremities
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• mice exhibit poor bone mineralization
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adipose tissue
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• mice lack adipose tissue
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cellular
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• mouse embryonic fibroblasts exhibit more misshapen nuclei than in wild-type cells
(J:113119)
• however, the frequency of misshapen nuclei is reduced following treatment with FTI (a farnesyltransferase inhibitor)
(J:113119)
• mouse embryonic fibroblasts exhibit more misshapen nuclei than in wild-type or Lmnatm1Lgf heterozygotes
(J:141165)
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growth/size/body
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• mice are half the size of wild-type mice
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craniofacial
Allelic Composition |
Lmnatm1Lgf/Lmna+
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Genetic Background |
involves: 129P2/OlaHsd * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm1Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
|
|
|
Lmnatm1Lgf/Lmna+ mice exhibt kyphosis, rib fractures, and skull abnormalities
mortality/aging
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• 50% of mice become malnourished and are euthanized by week 27
(J:113119)
• all mice die by 40 week of age
(J:113119)
• mice die by 35 weeks of age
(J:141165)
• mice die at 30 weeks
(J:146099)
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skeleton
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• cranial sutures have a zigzag appearance
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• by week 18, mice develop osteolytic lesions in the posterior portion of the zygomatic arch
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• mice develop osteolytic lesions in the ribs
(J:113119)
• ribs exhibit decreased bone density and cortical thickness compared to wild-type mice
(J:141165)
|
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• mice are pre-disposed to rib fractures near the costovertebral junction unlike wild-type mice
(J:113119)
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
(J:113119)
• mice develop progressive rib fractures near the costovertebral joints as they age
(J:141165)
|
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• mice develop kyphosis
(J:113119)
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
(J:113119)
|
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• mice exhibit osteolytic lesions on the ribs, zygomatic arch, clavicle, scapula, calvarium and mandible
|
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• ribs exhibit decreased bone density compared to wild-type mice
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• mice exhibit poor bone mineralization
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
|
adipose tissue
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• mice exhibit reduced subcutaneous adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
|
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• mice exhibit decreased abdominal adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
|
cellular
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• primary fibroblast lines that are heterozygous display increased nuclear blebbing, which is diminished by treatment with farnesyltransferase blocker PB-43
(J:100220)
• mouse embryonic fibroblasts exhibit misshapen nuclei
(J:141165)
• mouse embryonic fibroblasts exhibit misshapen nuclei unlike wild-type cells
(J:146099)
|
growth/size/body
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• mice begin to lose weight at 6 to 8 weeks of age
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
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behavior/neurological
N |
• unlike Zmpste24 knockout mice, grip strength is normal
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craniofacial
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• cranial sutures have a zigzag appearance
|
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• by week 18, mice develop osteolytic lesions in the posterior portion of the zygomatic arch
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integument
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• mice exhibit reduced subcutaneous adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
|
Allelic Composition |
Lmnatm1Lgf/Lmnatm1Stw
|
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Genetic Background |
involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 |
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm1Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Lmnatm1Stw mutation
(3 available);
any
Lmna mutation
(82 available)
|
|
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mortality/aging
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• mice die between 10 and 14 weeks of age
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skeleton
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• mice develop fractures in the extremities by 8 weeks of age
|