Phenotypes Associated with This Genotype

Genotype
MGI:3624987

• Allelic Composition: Tg(Ckm-LPL)MRze/0
• Genetic Background: involves: C57BL/6J * CBA/J

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

cellular

abnormal myocardial fiber mitochondrial morphology ( J:101818 )

• increase in the number of mitochondria in myocytes

mortality/aging

premature death ( J:101818 )

• die at 8-12 months of age

growth/size/body

decreased body weight ( J:101818 )

• gain weight slower than controls in the initial few weeks after birth and after 8 weeks of age, reach only 90% of the weight of controls

weight loss ( J:101818 )

• at 10 to 12 weeks of age, start to slowly lose weight

homeostasis/metabolism

decreased circulating triglyceride level ( J:101818 )

• 56.5% decrease in plasma triglyceride levels at 2-3 months of age and lower levels seen over the lifespan of mutants

decreased circulating VLDL triglyceride level ( J:101818 )

increased skeletal muscle glycogen level ( J:101818 )

• moderate increase in muscle glycogen deposition in vesicles or diffusely dispersed in the sarcoplasma at 5 months of age

increased muscle free fatty acids level ( J:101818 )

• 2.6-fold increase

abnormal enzyme/coenzyme activity ( J:101818 )

• 2 to 3-fold increase in succinated dehydrogenase activity

increased catalase activity ( J:101818 )

• 8-fold increase in catalase activity in skeletal muscle

muscle

increased muscle free fatty acids level ( J:101818 )

• 2.6-fold increase

abnormal muscle fiber morphology ( J:101818 )

• at 5 months of age, exhibit an increase in the number of mitochondria in the subsarcolemmic, perinuclear, and intermyofibrillar position of muscle fibers

abnormal myocardial fiber mitochondrial morphology ( J:101818 )

• increase in the number of mitochondria in myocytes

abnormal skeletal muscle morphology ( J:101818 )

• large increase in the number of peroxisomes in skeletal muscle

increased skeletal muscle glycogen level ( J:101818 )

• moderate increase in muscle glycogen deposition in vesicles or diffusely dispersed in the sarcoplasma at 5 months of age

myopathy ( J:101818 )

• at 5 months of age, exhibit a moderate loss of muscle tissue

adipose tissue

abnormal adipose tissue distribution ( J:101818 )

• at 5 months of age, exhibit a moderate loss of adipose tissue

cardiovascular system

abnormal myocardial fiber mitochondrial morphology ( J:101818 )

• increase in the number of mitochondria in myocytes

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
myopathy		DOID:423		J:101818