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Phenotypes Associated with This Genotype
Genotype
MGI:2177931
Allelic
Composition
Lmnatm1Stw/Lmnatm1Stw
Genetic
Background
involves: 129S1/Sv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm1Stw mutation (1 available); any Lmna mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all die by 8 weeks of age (J:58702)
• all die by 8 weeks of age (J:58702)

cellular
• in MEFs, nuclei appear elongated or irregular with loss of B-type lamins from 1 pole, slight clustering of nuclear pore complexes, and loss of emerin from the nuclear envelope into the cytoplasm (J:58702)
• in MEFs and hepatocytes, patchy thinning or loss of heterochromatin from the nuclear face of the inner nuclear membrane is seen and these regions also lack identifiable nuclear pore complexes (J:58702)
• the nuclear envelope is more easily fragmented during isolation (J:58702)
• in MEFs, nuclei appear elongated or irregular with loss of B-type lamins from 1 pole, slight clustering of nuclear pore complexes, and loss of emerin from the nuclear envelope into the cytoplasm (J:58702)
• in MEFs and hepatocytes, patchy thinning or loss of heterochromatin from the nuclear face of the inner nuclear membrane is seen and these regions also lack identifiable nuclear pore complexes (J:58702)
• the nuclear envelope is more easily fragmented during isolation (J:58702)

growth/size/body
• at 4 weeks, mean body weight is about 50% of wild-type and heterozygous littermates (J:58702)
• at 4 weeks, mean body weight is about 50% of wild-type and heterozygous littermates (J:58702)
• growth retardation is seen by 2-3 weeks of age and growth ceases by 4 weeks of age (J:58702)
• growth retardation is seen by 2-3 weeks of age and growth ceases by 4 weeks of age (J:58702)

muscle
• muscles surrounding the femur and perivertebral muscles are dystrophic while cardiac muscle is variably affected with ventricular muscle more severely impaired; however, muscles in the head, tongue and diaphragm are relatively normal (J:58702)
• muscle fibers are variably affected with those closer to the bone being more severely impaired (J:58702)
• no elevation of serum creatine kinase levels are detected (J:58702)
• muscles surrounding the femur and perivertebral muscles are dystrophic while cardiac muscle is variably affected with ventricular muscle more severely impaired; however, muscles in the head, tongue and diaphragm are relatively normal (J:58702)
• muscle fibers are variably affected with those closer to the bone being more severely impaired (J:58702)
• no elevation of serum creatine kinase levels are detected (J:58702)
• in the heart, some ventricular myocytes show signs of degeneration often associated with patchy mineralization (J:58702)
• in the heart, some ventricular myocytes show signs of degeneration often associated with patchy mineralization (J:58702)

behavior/neurological
• at 3-4 weeks mice have reduced grip strength (J:58702)
• at 3-4 weeks mice have reduced grip strength (J:58702)
• at 3-4 weeks mice display a stiff walking posture with splayed hind legs (J:58702)
• at 3-4 weeks mice display a stiff walking posture with splayed hind legs (J:58702)

skeleton
• progressive kyphoscoliosis starting around 3-4 weeks of age (J:58702)
• progressive kyphoscoliosis starting around 3-4 weeks of age (J:58702)

adipose tissue
• absence of white fat (J:58702)
• absence of white fat (J:58702)

nervous system
• sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are present (J:75378)
• sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are present (J:75378)
• sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are presen (J:75378)
• sciatic nerve axon density is reduced, axon diameter is increased, and nonmyelinated axons are presen (J:75378)
• nonmyelinated axons are present in the sciatic nerve (J:75378)
• nonmyelinated axons are present in the sciatic nerve (J:75378)

immune system
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)

hematopoietic system
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)

endocrine/exocrine glands
• probably secondary to physiological stress (J:58702)
• probably secondary to physiological stress (J:58702)


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory