Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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mortality/aging
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• treatment with an FTI increased the survival of both male and female mutants significantly compared with vehicle-treated mutant controls; by 20 weeks of age, 6 of 14 vehicle-treated mutants had died, but only 1 of 13 FTI-treated mutants was dead
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growth/size/body
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• male and female mutant mice treated with an FTI gain more weight than vehicle treated controls
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skeleton
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• at 20 weeks, the median number of rib fractures in FTO-treated mice is 2, compared to 14 in vehicle treated mice
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behavior/neurological
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• 100% of male and female mutant mice receiving vehicle exhibit abnormal grip strength by 16 weeks of age; only 28% of female and 33% of male mutants receiving a farnesyltransferase inhibitor (FTI) show a grip abnormality in a grid-hang test
• the delay in appearance of grip abnormalities is significant for male and female mutants
• the mean length of hang time of inhibitor-treated mice is longer than for vehicle treated mice
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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mortality/aging
skeleton
growth/size/body
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• far smaller than wild-type mice
(J:160705)
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behavior/neurological
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• invariably die from severe, progressive inanition
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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A Zmpste24tm1Sgy/Zmpste24tm1Sgy mouse.
mortality/aging
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• mice die or require euthanasia by 6-7 months of age
(J:79501)
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skeleton
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• thinner cortical bone and increased pock marking
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• the zigzag appearance of cranial sutures is reduced at 2-3 months of age
(J:79501)
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• unhealed fractures result in replacement of the posterior portion of the zygomatic arch with fibrous material containing bone spicules and necrotic debris
• however, at 18 days of age zygomatic arch morphology appears normal
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• at 3 months, lower incisors appear splayed apart, thin and long
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• at 2 to 3 months but not 18 days
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• by 24-30 weeks, nearly every rib is broken near the costovertebral junction and at the tip
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• bone density is reduced, bodies appear more porous, trabecular bone volume/total bone volume is reduced by 34%, and the thickness of trabeculae is reduced by 36%
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• bone density is reduced in thoracic vertebrae; however, plasma levels of calcium and phosphate are similar to wild-type and no change in bone turnover rate is detected
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• thinner cortical bone in the tibia and fibula even when corrected for bone size
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• in the thoracic vertebrae trabecular bone volume/total bone volume is reduced by 34%, and the thickness of trabeculae is reduced by 36%
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cellular
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• nuclear envelopes contain frequent blebs
(J:95274)
• MEFs display blebbing of the nuclear membrane
(J:106473)
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• striking accumulation of prelamin A in fibroblasts
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growth/size/body
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• at 3 months, lower incisors appear splayed apart, thin and long
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• at 6-8 weeks of age homozygotes appear very malnourished
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• weigh slightly less at weaning and gain weight slowly
(J:79501)
• severe growth retardation by 4 months of age
(J:106473)
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behavior/neurological
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• impaired grip strength by 4 months of age
(J:106473)
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• only able to hang from a wire grid for a few seconds
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• slow, hobbling gait with frequent dragging of the hindlimbs
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homeostasis/metabolism
muscle
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• muscle weakness that worsens with age; however no abnormalities are seen in skeletal muscle fiber morphology
(J:79501)
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adipose tissue
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• reduced but present, likely secondary to malnutrition
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cardiovascular system
N |
• no heart pathology is seen at any age
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liver/biliary system
N |
• no liver pathology is seen at any age
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limbs/digits/tail
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• thinner cortical bone and increased pock marking
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craniofacial
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• the zigzag appearance of cranial sutures is reduced at 2-3 months of age
(J:79501)
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• unhealed fractures result in replacement of the posterior portion of the zygomatic arch with fibrous material containing bone spicules and necrotic debris
• however, at 18 days of age zygomatic arch morphology appears normal
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• at 3 months, lower incisors appear splayed apart, thin and long
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• at 2 to 3 months but not 18 days
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integument
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• reduced but present, likely secondary to malnutrition
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Allelic Composition |
Zmpste24tm1Sgy/Zmpste24+
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Genetic Background |
involves: 129S4/SvJae * C57BL/6 |
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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skeleton
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• at 15 months of age in 2 of 2 heterozygotes degraded transverse and spinous processes are seen
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• at 15 months of age in 2 of 2 heterozygotes degraded transverse and spinous processes are seen
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growth/size/body
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• seen in older heterozygotes (around 15 months of age)
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behavior/neurological
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• appear weak at 15 months of age
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integument
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm6Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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digestive/alimentary system
N |
• mice do not exhibit intestinal abnormalities
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm10Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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digestive/alimentary system
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• esophagus shows a cornified squamous epithelium
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• mice develop dilated, yellow esophagus
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• thinning and fibrosis of the muscularis externa, pathology similar to achalasia
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muscle
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• thinning and fibrosis of the muscularis externa, pathology similar to achalasia
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nervous system
N |
• mice show no pathology in the central nervous system
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm6Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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mortality/aging
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• survival is worse than in Zmpste24 single mutants
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• survival is worse than in Zmpste24 single mutants
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growth/size/body
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• smaller than Zmpste24 single mutants
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm6Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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mortality/aging
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• survival is worse than in Zmpste24 single mutants
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• survival is worse than in Zmpste24 single mutants
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growth/size/body
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• smaller than Zmpste24 single mutants
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm7Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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mortality/aging
N |
• mice exhibit normal survival
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skeleton
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• compared to in Zmpste24tm1Sgy homozygotes
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growth/size/body
mortality/aging
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• die by 4-6 weeks of age
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growth/size/body
muscle
mortality/aging
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• die by 4-6 weeks of age
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growth/size/body
muscle
skeleton
N |
• no bone fractures or other skeletal abnormalities are seen
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cellular
N |
• normal nuclear envelope morphology in MEFs
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growth/size/body
N |
• improved growth compared to mice homozygous for Zmpste24tm1Sgy only
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muscle
N |
• muscle strength is similar to wild-type
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm2Lgf mutation
(0 available);
any
Lmna mutation
(82 available)
Zmpste24tm1Sgy mutation
(2 available);
any
Zmpste24 mutation
(32 available)
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skeleton
N |
• no bone fractures or other skeletal abnormalities are seen
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cellular
N |
• far fewer misshapen nuclei in MEFs
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growth/size/body
muscle
N |
• muscle strength is similar to wild-type
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