Phenotypes Associated with This Genotype

Genotype
MGI:3844908

• Allelic Composition: Large1^{enr-Tg(MbpReg)36Pop}/Large1^{enr-Tg(MbpReg)36Pop}
• Genetic Background: involves: C57BL/6J * DBA/2J

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

mortality/aging

premature death ( J:16281 )

• mice do not survive past 11 months dying between 6 and 8 months

postnatal lethality ( J:16281 )

• half as many mice as expected reach weaning; cause and time of death is unknown

reproductive system

reduced female fertility ( J:16281 )

♀ • few litters are delivered

male infertility ( J:16281 )

♂ • no successful matings occur

muscle

abnormal skeletal muscle morphology ( J:16281 )

• at 46 weeks, mice exhibit foci of calcification in the muscle unlike in wild-type mice

abnormal skeletal muscle fiber morphology ( J:16281 )

• muscle fibers lose their normal polygonal contour, nuclei are centrally located, and individual fibers vary in size unlike in wild-type mice

• older mice exhibit increased endomysial fibrous tissue and adipose tissue

skeletal muscle fiber necrosis ( J:16281 )

• with or without phagocytosis as early as 6 weeks of age

skeletal muscle endomysial fibrosis ( J:16281 )

behavior/neurological

abnormal posture ( J:16281 )

• by 3 to 4 months mice begin to display postural abnormalities unlike wild-type mice

• in some mice hindlimbs are drawn close together, splayed outward in a frog stance, or dragged one hindlimb stiffly behind them

abnormal limb posture ( J:16281 )

• mice display hindlimb stiffness

nervous system

abnormal myelination ( J:16281 )

• unmyelinated peripheral axons are observed unlike in wild-type mice associated with the absence of intervening Schwann cell cytoplasm between unmyelinated fibers

skeleton

kyphosis ( J:16281 )

• in the thoracic region

growth/size/body

decreased body size ( J:16281 )

• at 2 to 3 weeks of age