Phenotypes associated with this allele

• Allele Symbol: Gt(ROSA)26Sor^{tm1(CAG-LMNA*)Cyh}
• Allele Name: targeted mutation 1, Ya-Hui Chi
• Allele ID: MGI:6407423
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Gt(ROSA)26Sor^tm1(CAG-LMNA*)Cyh/Gt(ROSA)26Sor^+ Tg(Ckmm-cre)5Khn/0	involves: C57BL/6JNarl * FVB	MGI:6407437

Genotype
MGI:6407437

cn1

• Allelic Composition: Gt(ROSA)26Sor^{tm1(CAG-LMNA*)Cyh}/Gt(ROSA)26Sor⁺; Tg(Ckmm-cre)5Khn/0
• Genetic Background: involves: C57BL/6JNarl * FVB

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:284048 )

• median lifespan of 40 days

growth/size/body

decreased body fat mass ( J:284048 )

♂ • net volume and ratio of fat mass is lower

decreased body size ( J:284048 )

• adults (2 months of age) appear smaller, with decreased size beginning at 3 weeks after birth

• body weight of 2 month old males is about 35% lower than in controls

muscle

abnormal skeletal muscle morphology ( J:284048 )

• skeletal muscle shows dilated endoplasmic reticulum lumen, reduced mitochondrial cristae density, and ruffled nucleus morphology

decreased skeletal muscle fiber diameter ( J:284048 )

• reduction in muscle cross-sectional area of the soleus muscle

centrally nucleated skeletal muscle fibers ( J:284048 )

• increase in the prevalence of central nuclei in the myofibrils of the soleus muscle

decreased skeletal muscle mass ( J:284048 )

• reduction in muscle mass

dystrophic muscle ( J:284048 )

adipose tissue

decreased body fat mass ( J:284048 )

♂ • net volume and ratio of fat mass is lower

abnormal white adipose tissue morphology ( J:284048 )

♂ • cell volume of white adipose tissue is lower

♂ • however, histology of brown adipose tissue is unaffected and lean mass is unaffected

behavior/neurological

jerky movement ( J:284048 )

♂ • frequency of twitching is increased

decreased grip strength ( J:284048 )

♂ • 50% reduction in grip strength

decreased locomotor activity ( J:284048 )

♂ • mice show reductions in the frequencies of drinking, hanging, rearing and walking during the night indicating that mice are less active at night

♂ • however, no differences in daytime activity are seen

cardiovascular system

decreased heart rate ( J:284048 )

prolonged QT interval ( J:284048 )

cellular

abnormal cell nucleus morphology ( J:284048 )

• nuclear morphology of skeletal muscle becomes ruffled

abnormal endoplasmic reticulum morphology ( J:284048 )

• endoplasmic reticulum lumen in the myonuclei is dilated

abnormal mitochondrial crista morphology ( J:284048 )

• density of mitochondrial cristae is reduced is skeletal muscle

increased endoplasmic reticulum stress ( J:284048 )

• marker analysis indicates elevated ER stress in skeletal muscle

homeostasis/metabolism

increased circulating creatine kinase level ( J:284048 )

decreased body temperature ( J:284048 )

♂ • surface temperature of the eye and the trunk is lower indicating lower body temperature

decreased carbon dioxide production ( J:284048 )

♂ • carbon dioxide production per unit activity is lower during the night

decreased oxygen consumption ( J:284048 )

♂ • whole-body consumption of oxygen is lower during the night

increased respiratory quotient ( J:284048 )

♂ • the respiratory exchange ratio is increased during the day

decreased basal metabolism ( J:284048 )

♂ • heat production is lower at night but not during the day

skeleton

kyphosis ( J:284048 )

• adults present kyphosis