About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Lmnatm1Lgf
targeted mutation 1, Loren G Fong
MGI:3587791
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Lmnatm1Lgf/Lmnatm1Lgf involves: 129P2/OlaHsd * C57BL/6 MGI:3817509
ht2
Lmnatm1Lgf/Lmna+ involves: 129P2/OlaHsd * C57BL/6 MGI:3817506
ht3
Lmnatm1Lgf/Lmnatm1Stw involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 MGI:3817521


Genotype
MGI:3817509
hm1
Allelic
Composition
Lmnatm1Lgf/Lmnatm1Lgf
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)
phenotype observed in females
phenotype observed in males
N normal phenotype

Lmnatm1Lgf/Lmnatm1Lgf mice are small and have poorly mineralized bone

mortality/aging
• all mice die by 3 to 4 weeks of age

skeleton
• mice develop fractures in the extremities
• mice have open sutures
• mice exhibit poor bone mineralization

adipose tissue
• mice lack adipose tissue

cellular
• 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)

growth/size/body
• mice are half the size of wild-type mice

craniofacial
• mice have open sutures




Genotype
MGI:3817506
ht2
Allelic
Composition
Lmnatm1Lgf/Lmna+
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)
phenotype observed in females
phenotype observed in males
N normal phenotype

Lmnatm1Lgf/Lmna+ mice exhibt kyphosis, rib fractures, and skull abnormalities

mortality/aging
• 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)

skeleton
• cranial sutures have a zigzag appearance
• by week 18, mice develop osteolytic lesions in the posterior portion of the zygomatic arch
• mice develop osteolytic lesions in the ribs (J:113119)
• ribs exhibit decreased bone density and cortical thickness compared to wild-type mice (J:141165)
• 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)
• mice develop kyphosis (J:113119)
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype (J:113119)
• mice exhibit osteolytic lesions on the ribs, zygomatic arch, clavicle, scapula, calvarium and mandible
• ribs exhibit decreased bone density compared to wild-type mice
• mice exhibit poor bone mineralization
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype

adipose tissue
• mice exhibit reduced subcutaneous adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype
• mice exhibit decreased abdominal adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype

cellular
• 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
• mice begin to lose weight at 6 to 8 weeks of age
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype

behavior/neurological
N
• unlike Zmpste24 knockout mice, grip strength is normal

craniofacial
• cranial sutures have a zigzag appearance
• by week 18, mice develop osteolytic lesions in the posterior portion of the zygomatic arch

integument
• mice exhibit reduced subcutaneous adipose tissue compared to wild-type mice
• however, treatment with FTI (a farnesyltransferase inhibitor) improves phenotype

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
progeria DOID:3911 OMIM:176670
J:113119




Genotype
MGI:3817521
ht3
Allelic
Composition
Lmnatm1Lgf/Lmnatm1Stw
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)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die between 10 and 14 weeks of age

skeleton
• mice develop fractures in the extremities by 8 weeks of age





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
04/23/2024
MGI 6.23
The Jackson Laboratory